Von Hippel-Lindau syndrome is characterized by tumors as central nervous system and retinal hemangioblastomas; renal, liver and pancreas cysts; clear cells renal carcinoma, pheochromocytoma and adenomas of the ear, nose and larynx. In most cases there is a positive family history of the syndrome 8. The follow case is about a female how was 35 years old without known medical history. She had a positive family history of von Hippel-Lindau, and her family told us about she had “stiffness attacks” and two weeks later had another stiffness attack so it was treated by paramedics who declared her dead. In agreement with the forensic autopsy findings the diagnosis was hydrocephalus induced by brain stem hemangioma and the findings were compatible with von Hippel-Lindau. The aim of this article is to highlight the most important postmortem findings of this syndrome and its relation to the cause of death.

Non traumatic spleen rupture; surgical instrumentation; multiple transfusions; bleeding on massive transfusions