Abstract:Pseudomyxoma peritonei is rare, chronic, relapsing, diagnostically challenging disease which is characterized by disseminated mucinous ascites. The presence of inflammatory or neoplastic cells in mucin distinguishes it from simple ascites. It is mainly due to complication of borderline or malignant neoplastic lesion of appendix and/or ovary. The clinical outcomes vary in different histological types and corresponding treatment modalities. Most of the clinicians think that surgical debulking with appendectomy is the line of treatment but this is still controversial as the role of chemotherapy is still being evaluated. We report a case of 50 years female and review unique clinical features and the misconceptions surrounding pseudomyxoma peritonei.