Pseudomyxoma peritonei is an unusual condition more common in females, in which massive amounts of mucinous ascites in conjunction with mucinous peritoneal and omental implants occur. We performed a MEDLINE search of the English-language literature from 1966 to June 2000, utilizing the key words pseudomyxoma peritonei. All case reports, series and studies regarding this condition were reviewed. Cross-referencing was also performed. The etiology and treatment of this condition are currently controversial. Most investigators agree that surgical debulking and appendectomy are adequate initial therapeutic measures. The role of intraperitoneal chemotherapy, radiotherapy or application of mucolytic therapy remains uncertain. Recent molecular genetic studies suggest the appendix as the organ of primary origin of this disease.