Acute hemorrhagic enteritis was diagnosed in a seven-month-old male Shitzu dog dying of blood stained diarrhea and vomiting. Clinical findings were anorexia, dullness and sudden death after massive bloody diarrhea. At necropsy, main lesion was the hemorrhage in small intestine, mainly duodenum and jejunum. Microscopically, Gram positive long bacilli were massively detected on the mucose epithelial cells and necrotic debris of small intestine. Coagulative necrosis of epithelial cells and thrombosis of small intestine were also identified. However, there was no lesion of crypt epithelium. Mineral infiltration in both gastric mucosa and renal tubules was detected and proliferation of fibrous tissue was also shown in corticomedullary regions. In bacterial examination, C perfringens was isolated in anaerobic culture and it was confirmed to type A by multiplex PCR. Therefore, the dog was diagnosed as C perfringens type A associated enteritis with uremia.