Background: Gonodoblastomas with ovarian germ cell tumors (OGCTs) other than dysgerminoma are very unusual. This is the first case report of a pure endodermal sinus tumor of the ovary with gonodoblastoma in a 46 XX, female. Case: A 19-year-old female was admitted to our hospital with an abdominal mass and pain. She had elevated levels of CA-125 and AFP Fertility sparing surgery was undertaken and revealed a Stage IA endodermal sinus tumor and gonodoblastoma in the same ovary. The patient refused chemotherapy and was followed-up for six months without disease. Conclusion: Gonodoblastomas may occur in conjunction with OGCTs. Tumor markers and immunohistochemical examination may help in the diagnosis of these OGCTs with gonodoblastoma.