Pseudomyxoma peritonei (PMP) is a rare disease with an incidence of two per million. Acute appendicitis, ovarian mass, and abdominal distension are the most common presentations. A 72-year-old male patient with a history of laparoscopic cholecystectomy was admitted to the hospital with abdominal pain and increased supraumbilical port site swelling. Radiological examination revealed a mass in the terminal ileum and severe intraabdominal mucinous fluid. Intraabdominal gelatinous fluid protruding from the port site defect and a mass in the distal appendix were observed during operation. He underwent a right hemicolectomy, ileocolic anastomosis and peritoneal debridement; cytoreductive surgery was administered two months later due to mucinous tumor of the appendix. There are only a few case reports describing PMP presenting with an incisional hernia after open surgical procedures. To the best of our knowledge, this report describes the first case of PMP presenting with a port site hernia after a laparoscopic intervention.