Pseudomyxoma peritonei (PMP) is an uncommon clinical syndrome that can present as an ovarian mass in women, mimicking ovarian malignancy. PMP predominantly originates from a primary appendiceal neoplasm, with a spectrum of disease aggressiveness usually correlating with histological findings. Therefore, PMP is, at best, a borderline malignancy. Management and outcome of patients with PMP have improved over the past three decades with better understanding and awareness of optimal cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy. Challenges remain in achieving complete cytoreduction and in the management of patients with recurrence after complete cytoreduction.