Splenic artero-venous fistula (SAVF) is a rare but potentially curable cause of pre-hepatic portal hypertension. About 100 cases have been reported in the world medical literature. The Authors report a case of 46-year-old man with a splenic artery aneurysm and a large SAVF treated by surgical resection of splenic vessels and splenectomy. The literature about SAVF is reviewed to recognize etiology, anatomical location, main symptoms at presentation, diagnostic findings and management of this rare syndrome.