SUMMARY -The authors present the autopsy findings of two related patients and the biopsy findings of a thrid member of the family. The oldest member was 34 years old at death and on postmortem examination he had haemangioblastomas in the retina, cerebellum, medulla and spinal cord. Other findings were renal cell carcinoma, KEY WORDS: von Hippel-Lindau's disease, angiomatosis, phakomatosis. Doença de von Hippel-Lindau: relato de três casos e revisão da literaturaRESUMO -Os autores relatam os achados de autópsia de dois pacientes de uma mesma família e o diagnóstico por biópsia de hemangioblastoma de um terceiro membro desta mesma família. O primeiro paciente tinha 34 anos por ocasião do óbito e os achados de necrópsia mostraram hemangioblastoma de retina, cerebelo, bulbo e medula espinhal, além de carcinoma renal, feocromocitoma, lesões císticas de rim e pâncreas, hidromielia e meningiomas atípicos. Seu irmão morreu com 30 anos de idade e a autópsia revelou hemangioblastomas de cerebelo, carcinoma renal e cistoadenoma de células claras de epididimo. A terceira paciente era filha do primeiro PALAVRAS-CHAVE: doença de von Hippel-Lindau, angiomatose, facomatose.Von-Hippel-Lindau's disease (vHL) is a rare autosomal dominant disorder with incomplete penetrance and variable expression affecting 1:100000 inhabitants initially described by Turner in 1887 1719 . It was characterized as a clinical entity by Lindau (1926) who described the occurrence of central nervous system and visceral abnormalities including haemangioblastomas of the cerebellum, retina and spinal cord, pancreatic and renal cell carcinoma and cysts, and phaeochromocytoma 5 .In the present study we present the post-mortem findings of two familial cases and a further case of the same family operated by cerebellar haemangioblastoma; so far no similar reports have been documented in the Brazilian literature.