The clinical manifestations of von Hippel and von Hippel-Lindau's disease are illustrated. Four cases are presented with special emphasis on the angiographic characteristics, the evolution and the complications of the retinal capillary hemangiomas. Special attention is given to fundoscopy, fluorescein and indocyanine green angiography and to the systemic manifestations. We summarize new molecular genetic insights. The importance of systemic and familial evaluation is stressed. Retinal angiomatosis is a clinical diagnosis but the angiography has an adjuvant value.