Summary Objective To study the pattern of occurrence of Creutzfeldt–Jakob disease (CJD) in Kenya. Study design Prospective, cross-sectional, descriptive study of clinical, encephalographic and natural history of CJD, backed by histology in as many patients as possible. Methods Consecutive patients presenting with the criteria laid down by WHO expert committee for diagnosis of CJD were recruited between January 1990 and May 2004. We analysed the clinical features and electroencephalography of all participants and took brain biopsies from four patients. Results There were four definite, seven probable and two possible cases. The electroencephalographic and histological features were typical of sporadic CJD. Conclusion Sporadic CJD occurs in Kenya and the clinical, encephalographic and histological features were no different to those described elsewhere. Although we did not see variant, hereditary and iatrogenic forms of CJD, neurologists should not exclude these in making diagnoses.