Abstract
Portal venous aneurysm is a rare and potential dangerous vascular pathology, which can result in thrombosis or rupture. It may be congenital or acquired. Acquired form can be related mainly to portal hypertension, chronic hepatic disease, and trauma. We present a peculiar case of a congenital aneurysm involving the hepatic portal system in nearly all its extra-hepatic components: the main portal trunk, the spleno-porto-mesenteric confluence and the distal segment of splenic, superior, and inferior mesenteric veins, in a 20-year-old male patient. The aneurysm was complicated by massive thrombosis in absence of further predisposing factors.