Pseudomyxoma peritonei (PMP) is a rare, progressive disease with extensive mucinous accumulation within the peritoneal cavity. It originates from low malignant potential tumours of the appendix or ovaries. Different biological behaviour of tumours of various sites leads to a dilemma in diagnosis and management. The computed tomography (CT) scan is the preferred imaging modality in selection of patients for operation and for follow-up. The therapy of pseudomyxoma peritonei is palliative treatment, which consists in multiple cytoreductive surgery and sometimes in hyperthermic intraperitoneal chemotherapy. These methods have reduced mortality and morbidity. Although the disease is minimally invasive, the expected long-term survival is limited with no prospect of cure. This paper describes a case of pseudomyxoma peritonei and presents the available literature data.