Objective: Pseudomyxoma peritonei (PMP) is a rare, chronic, relapsing, diagnostically challenging, and poorly understood disease characterized by disseminated mucinous ascites and peritoneal implants. PMP usually develops secondary to mucinous neoplasms of the appendix and/or the ovary. Only few reports of limited PMP were published. Case Report: We describe the case of a 53-year-old woman who presented with a limited mucinous collection in the pelvis seen on computed tomography and who underwent surgery for mucocele of the appendix, bilateral mucinous ovarian tumors, and single mucinous implant on the great omentum 16 years previously. Serum tumor markers: CEA was elevated and CA 19-9 was within the normal range. At surgery, a well-circumscribed collection of mucin was found in the pelvis, and removal of mucinous material with intraoperative hyperthermic intraperitoneal chemotherapy (HIPEC) and postoperative intraperitoneal irrigation with 5-fluorouracil was performed. Histological examination revealed disseminated peritoneal adenomucinosis according to Ronnett’s classification. After surgery, the patient has been free of recurrence for 2 years. Conclusion: Our case report highlights that PMP requires careful observation, as it might be detected after a long-term follow-up period post-appendectomy for appendiceal mucocele.