Pseudomyxoma peritonei (PMP) is rare clinical entity presenting with mucus-producing lesions on the peritoneal surface. PMP is found in middle-aged or older patients in approximately two out of 10,000 laparotomies. Most of the reported cases are due to a primary process located in either appendix or the ovaries. PMP presents with an insidious onset of symptoms, and is characterized by long-term survival with good general health and absence of visceral invasion or distant metastasis. The treatment of choice for PMP is surgical resection and the removal of free mucus. Despite a high rate of disease recurrence, adjuvant or palliative chemotherapy has not yet been clearly established. In our opinion, intraperitoneal application may be more effective than a systemic regimen because only local treatment can provide sufficient concentrations of chemotherapeutic agents. In view of the lack of standardized treatment regimens and based on our experience, we recommend intraperitoneal chemotherapy with 5-FU and mitomycin C or cisplatin. We report ten cases of PMP of appendiceal origin and present a review of the literature on this disease and its treatment.