Pseudomyxoma peritonei is a rare disease characterized by a complete redistribution of mucinous ascites into the peritoneal cavity. We report a case of pseudomyxoma peritonei in a 68 year old woman. In July 2005 she underwent surgery elsewhere. Only partial resection of the great omentum was performed. Adjuvant therapy was also administered (Folfox 4, four cycles). Six months later the same peritoneal carcinomatosis was detected by CT. In December 2005 the tumor was removed en bloc by performing left hemicolectomy, anterior resection of the rectum, hystero-adnexectomy, right hemicolectomy, splenectomy, omentectomy, total peritonectomy, hepatic resection (segment IV), cholecystectomy, pericaval, portal and iliac lymphadenectomy, colorectal anastomosis, ileal-colon anastomosis and protective ileostomy. Surgery was completed by intraperitoneal hyperthermic chemotherapy consisting in Cisplatin 200 mg and Mitomycin C 30 mg, administered in 6500 cc of perfusion liquid for 60 minutes at a temperature of 42.5 degrees Celsius, by means of the closed technique. The only noteworthy postoperative complication was a left ureteral fistula appearing on the 20th postoperative day and treated by placement of a ureteral stent. Histological and immunohistochemical examinations: pseudomyxoma peritonei. The most recent literature shows excellent long-term survival if the disease is treated combining peritonectomy and HIIC.