Abstract
A 61-year-old man was admitted to our hospital complaining of acute abdominal pain. Abdominal enhanced CT scan revealed the thrombosis of the superior mesenteric vein as well as air-gas in the intestinal wall. He was diagnosed to have small intestinal necrosis and underwent surgical resection of 120 cm of the ileum. The marginal vein of the necrotic ileum was obstructed by thrombosis. The postoperative laboratory data showed a remarkable deficit of protein S activity. In our case, small intestinal necrosis was caused by the superior mesenteric vein thrombosis associated with protein S deficiency.