BACKGROUND: Malignant rhabdoid tumors are rare, aggressive neoplasms that consist of both renal and extrarenal subtypes. Although extrarenal rhabdoid tumors have been documented at multiple extrarenal sites, to our knowledge no primary ovarian cases have been reported. CASE: An 18-year-old, Caucasian woman was diagnosed with a pure primary extrarenal rhabdoid tumor of the ovary following diagnostic laparoscopy for pelvic pain. The tumor exhibited rapid growth, failed to respond to chemotherapy and led rapidly to death. CONCLUSION: Although no other reports on primary ovarian extrarenal rhabdoid tumor have been published, the aggressive behavior of the tumor in this patient was similar to that seen in patients with metastatic disease.