Pseudomyxoma peritonei (PMP) syndrome is an uncommon, slowly progressive condition that usually arises from perforation of an adenoma in the appendix. PMP syndrome is characterized by mucin accumulation in the peritoneal cavity. Mucinous implants are found on all peritoneal surfaces and the omentum. PMP syndrome rarely metastasizes outside the abdominal cavity but remains a fatal illness as the space in the abdomen and pelvis required for normal function of the gastrointestinal tract becomes filled with copious amounts of the mucinous tumor. Treatment options include observation, aggressive debulking surgery, intraperitoneal chemotherapy, radiotherapy, and mucolytic agents.