Abstract
A 31-year-old woman presented to King Abdulaziz University Hospital complaining of an abdominal pain and a rapid increase in abdominal girth. An ultrasound and MRI, revealed a huge cystic ovarian mass without ascites. Ovarian tumor markers were all within normal range. Exploratory laparotomy showed huge right ovarian mass with omental mass. Frozen section from the omentum showed metastatic malignant neoplasm. Total abdominal hysterectomy was carried out with bilateral salpingooophorectomy and omentectomy with residual tumor of less then one centimeter. Final pathology assessment showed primitive neuroectodermal tumor arising from the right ovary. She received post-operative chemotherapy. Four months later she had recurrence and was given second line chemotherapy, but she did not respond and died 15 months after the diagnosis due to obstructive uropathy.