Introduction: Primary ovarian lipoleiomyoma is a rare benign tumor of the ovary predominantly consisting of smooth muscles and adipocytes. Clinically, they are asymptomatic and are diagnosed accidentally during the histopathological examination after the surgery.Case presentation: Herein, we report a case of unilateral, primary ovarian lipoleiomyoma in a 20-year-old primigravida who presented with complaints of abdominal distension for 4 months. The abdominal contrast-enhanced computed tomography (CECT) revealed a large, well-de ned, heterogeneously enhancing mass in the right adnexa measuring 35.9x26.1x12.9 cm. A primary clinical diagnosis of teratoma was raised. Unilateral salpingo-oophorectomy was performed, and histomorphology revealed a benign smooth muscle tumor admixed with mature adipocyte and cartilage arising primarily in the ovary. The diagnosis was con rmed by immunohistochemistry. The tumor may be asymptomatic or manifest with abdominal pain or distension, like in our index case. As there are no pathognomonic clinical symptoms, tumor markers, or characteristic imaging ndings, de nitive diagnosis is di cult prior to surgical removal. Histomorphology and immunohistochemistry con rm the smooth muscle nature of the tumor.Conclusion: This common and benign solid tumor at an unusual location should be kept in the differential diagnosis of solid ovarian masses.