Chandra Bahadur Dangi, verified by the Guinness Book of Records as the world's shortest man - at 21.5 inches (54.6cm) - died of an undisclosed illness in American Samoa.
The shortest man shot to fame after his pictures with the world's tallest man, Sultan Kosen from Turkey, were released in 2013. He was touring Samoa Islands, a US territory in South Pacific Ocean, when he fell sick and was admitted to Lyndon B Johnson Tropical Medical Center in Pago Pago at around midday.
Thaneswar Guragai, who assisted him on his tours, was quoted in Himalayan Times: "Chandra Bahadur Dangi's nephew, Dolakh, called me from the Samoa Islands and asked me to inform the family about the death." Guragai was in Kathmandu when Dangi breathed his last.
The 75-year-old Dangi lived in a remote village around 400km (248 miles) from Kathmandu, and travelled the world after he was officially declared the world's smallest man in 2013.
Besides being declared as the shortest man, Dangi also had a record of the world's shortest adult ever measured. Dangi was the seventh sibling of a family of six brothers and two sisters, all of whom are normal in height.
The medical centre in Samoa Islands where Dangi was undergoing treatment, is preparing to issue the death certificate before handing over his body to his family or their representative, he added.