Purpose: Pseudomyxoma peritonei (PMP) is a rare condition with inevitable persistence and progression. Although its etiology is controversial, commonest site of the origin is the appendix and the ovary. In this study, our purpose is to discuss PMP and to share our experience. In our clinic, two patients, a male and a female with a diagnosis of PMP were operated. Two patients died because of tumor recurrences. Even though PMP is not considered aggressive biologically and there is not a lymph node metastasis and invasion, it is eventually fatal with recurrences and progression of the disease. Due to rarity and heterogenicity of the condition, the treatment of PMP is subject of discussion.