We recently treated a patient with strangulating intestinal necrosis caused by the enlarged abdominal cysts characteristic of autosomal dominant polycystic kidney disease (ADPKD). A 75-year-old Japanese woman with a 30-year history of ADPKD underwent surgery to treat a diagnosis of acute abdomen. Surgery confirmed that the mesenterium was stretched caudally over the huge cysts that occupied the entire abdominal cavity, and the intestines were necrotic from the jejunum to the transverse colon. Veins from the necrotic intestine were dilated, and an arterial pulse could be felt. These findings resulted in a diagnosis of strangulation necrosis of the intestine caused by occlusion of the mesenteric vein by the enlarged cysts. The necrotic intestine was excised, and a proximal jejuno-transverse colostomy was performed. Pathological examination revealed hemorrhagic necrosis and marked congestion of the resected intestine. This case describes a rare, but clinically important, variation of ADPKD.