Pseudomyxoma peritonei is characterized by mucinous ascites and implants throughout the abdominal cavity. Although not a typical malignant entity the disease shows a long-term poor prognosis, because mucinous implants cannot be resected radically. Additional therapy is not able to improve the prognosis. The paper presents a case of a 58-year-old male, suffering fom a perforated mucocele with subsequent pseudomyxoma peritonei as well as a literature review about additional forms of therapy which might improve the disease together with a surgical approach.