Abstract Primary neoplastic lesions presenting with a mucocele of the Appendix are very rare and can be divided in to benign variant of mucinous adenomas or cyst adenomas, mucinous tumours of uncertain malignant potential or mucinous cyst adenocarcinomas. Most of these tumors are asymptomatic and are found incidentally or by symptoms of simple appendicitis. We describe an unusual presentation of pseudomyxoma peritonei( the major complication of neoplastic mucinous appendiceal tumor ) with urologic symptoms