Pseudomyxoma peritonei is a rare form of mucinous ascites associated with peritoneal and omental implants. The origin is controversial, and recent immunohistochemical and molecular genetic evidence suggests the appendix to be the likely site. The condition often presents as an incidental finding at laparotomy. Ultrasonography, computed tomography and magnetic resonance imaging aid in preoperative diagnosis. Treatment remains controversial, surgery being the main stay. The role of intraperitoneal and systemic chemotherapy is poorly defined. We review the literature on the pathology, clinical features and treatment options in pseudomyxoma peritonei.