Kuru, the first human transmissible spongiform encephalopathy was transmitted to chimpanzees by D. Carleton Gajdusek (1923–2008). In this review, I briefly summarize the history of this seminal discovery along its epidemiology, clinical picture, neuropathology and molecular genetics. The discovery of kuru opened new windows into the realms of human medicine and was instrumental in the later transmission of Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease as well as the relevance that bovine spongiform encephalopathy had for transmission to humans. The transmission of kuru was one of the greatest contributions to biomedical sciences of the XX century. “Kuru” in the Fore language of Papua New Guinea means to tremble from fever or cold. Kuru was restricted to natives of the Fore linguistic group in Papua New Guinea’s Eastern Highlands and neighbouring linguistic groups. Ritualistic endocannibalism (eating of relatives as part of a mourning ritual in contrast to eating enemies, i.e. exocannibalism) was practiced not only in the kuru area but in many surrounding Eastern Highland groups in which kuru never developed. The first who formally published the hypothesis that kuru spreads through cannibalism was Lindenbaum and Glasse.