A woman who was being treated for rabies in London has died, University College London Hospitals NHS Foundation Trust said on Monday.
She was being treated at London's Hospital for Tropical Diseases.
A statement from University College London Hospitals NHS Foundation Trust said: "We regret to announce that a patient being treated for rabies by the Hospital for Tropical Diseases and colleagues at University College Hospital died over the weekend.
"The patient's family have kindly requested for the media to respect their privacy during this very difficult time and we will not be releasing any further details, nor will the family be making any statements.
"Our sincere condolences go out to them."
Rabies is usually transferred through saliva from the bite of an infected animal, with dogs being the most common transmitter of rabies to humans.
More than 55,000 people are estimated to die from the disease every year, with most cases occurring in developing countries, particularly South and South-East Asia.
The hospital previously reassured patients, visitors and staff there was no risk to them as a result of the case.
An investigation was launched into how the woman was reportedly turned away from the hospital emergency department.
A spokesman for Darent Valley Hospital last week said: "The UK is rabies-free.
"If a patient does present at hospital with vague symptoms, a doctor is unlikely to consider rabies as a diagnosis unless the patient highlights wild animal contact in an at-risk country.
"The hospital responded to the information supplied by the patient at the time.
"Although there are no cases of rabies being passed through human-to-human contact, the five members of staff that came into close contact with the patient are being vaccinated as a precautionary measure.
"We have launched an investigation into the circumstances around this lady's attendance at the emergency department and we are working closely with the Health Protection Agency (HPA)."
On Friday disease control experts announced a second possible British case of rabies was being investigated in the case of a patient from Leeds.
The patient sought medical attention following a dog bite abroad, the Health Protection Agency said.
The HPA said the patient has "no links" to the confirmed case in London.
A spokeswoman said: "The HPA has been notified that a patient from Leeds has sought medical attention following a dog bite which occurred abroad.
"Investigations are under way to determine if the bite has led to an infection. There are no links to the rabies case confirmed recently in London."
Dr Ron Behrens, travel medicine expert at the London School of Hygiene and Tropical Medicine, said the prognosis was "bleak" for people infected with rabies, with only one or two cases known to have survived rabies encephalitis - the disease once it reaches the brain.
But he urged anyone who had been bitten by an animal they thought might have rabies to seek medical advice.
Dr Behrens said symptoms resulted from inflammation of the brain from the virus.
"In the early stages, the symptoms are mild headaches, some anaesthetic feeling around the site of a bite and fever.
"There is often confusion and delirium - disorientation often associated with abnormal and sometimes violent behaviour."
He said a unique symptom is a fear of water, which means patients cannot drink or swallow.
He said they ultimately become comatose during the terminal stages.
"The prognosis is bleak as most patients with rabies encephalitis die," Dr Behrens said.
"Only one or two cases are known to have survived the disease.
"Once the patient has developed encephalitis, the management involves making the patient comfortable by sedation and paralysing them and placing them in an anaesthetic coma.
"At this stage, none of the vaccines or immunoglobulin has any benefit."
Dr Behrens said rabid dogs in their early stages do not behave unusually and the disease can incubate from weeks to years after infection with no signs.
If bitten by a rabid dog, there is roughly 24 hours where treatment with an antibody can prevent the virus entering the nervous system, he said.