A rare case of acute abdomen syndrome due to a ruptured ovarian tumor and a penetrated fallopian tube is described. Based on the intraoperative finding and patient's age, a right-sided salpingo-oophorectomy, appendectomy and peritoneal washings were performed. After a histological diagnosis of malignant granulosa cell tumors and FIGO IIA clinical classification, the patient returned 1 month after the procedure. A relaparotomy with a hysterectomy, left-sided salpingo-oophorectomy and omentectomy were then performed. No spread was found during the laparotomy, and the histologic diagnosis pointed to adenomyosis and chronic cervicitis. The patient regularly presents for control examination, and has now been free for 11 years since the surgery from clinical, biochemical or ultrasonographic signs of a relapse of the disease.