Hemangioendothelioma is seldom seen in adults. Its severe evolution is due, not so much to the exceptional transformation into hemangiosarcoma, but mainly to haemorrhagic complications by rupture or consumption coagulopathy and to severe cardiac insuffficiency secondary to arteriovenous shunts. The case reported here concerns a 64 year-old woman presenting pain in the left hypochondrium and splenomegaly. A splenectomy was performed and the histological findings were compatible with the diagnosis of hepato-splenoganglionic hemangioendothelioma. The evolution was unfavorable. The patient died a few months later in a picture of haemorrhagic syndrome and cardiac insufficiency. Histological findings on autopsy specimens indicated a cavernous hemangioma. The treatment of these diffuse hemangiomas is a difficult one. Hepatic artery ligation has been advocated in certain desperate situations. Nevertheless, because of a collateral circulation, recurrences are frequent.