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This is a case report of primary appendiceal carcinoma presenting with pseudomyxoma peritonei.<BR>A 52-year-old male presented with a tender mass in the right lower quadrant of the abdomen, increasing in size. Barium enema revealed no abnormalities. Abdominal ultrasonography revealed a hypoechoic mass with multiple small septations corresponding to the soft mass spreading over the umbilicus. A CT-film showed a tumor with irregular density just below the abdominal wall and ascites. By cytological evaluation of ascitic fluid, pseu-domyxoma peritonei was suspected without definite evidence of mailgnancy. At operation, a cystic mass, about 3 cm in size, was found at the tip of the appendix and the peritoneal cavity was filled with a large amount of gelatinous material. Microscopically, the diagnosis of the resected appendix was cystic adenocarcinoma with psendomyxoma peritonei.<BR>Generally speaking, the diagnosis of primary appendiceal carcinoma is very difficult preoperatively and the authors also felt the difficulty in establishing a correct diagnosis.