To report on the safety of conservative management of a young woman with pseudomyxoma peritonei of appendiceal origin desiring fertility preservation. Case report. IVF Center at Tertiary Hospital. A 32-year-old woman with primary infertility. Appendectomy, conservative second-look laparoscopy, and IVF. Persistence of active mucinous lesions in the peritoneal cavity, presence of abnormalities at colonoscopy, and maternal/fetal complications from IVF. Normal second-look laparoscopy, no evidence of persistent disease, and successful intrauterine twin pregnancy following IVF. Pseudomyxoma peritonei of appendiceal origin is rare in young female patients. Although traditional therapy in older patients often involves aggressive surgical therapy, this case report demonstrates that patients desiring fertility can be managed conservatively and safely and still maintain successful pregnancy.