Background: Angioleiomyoma (ALM) is a rare, benign neoplasm involving the peripheral soft tissues. ALM has not previously been described to originate from the ovary. We present a case herein of a premenarchal girl with a large ALM originating from the ovary. Case: An 11-year-old girl underwent a laparotomy during the course of evaluation and treatment of a palpable, painless, abdominal mass. The mass was shown to have a strong blood flow by sonography. A solid mass left ovarian was identified and resected. The final pathologic report of the resected tumor was consistent with an ALM. Conclusion: To our knowledge, this is the first report of an ALM arising from the ovary. The treatment of choice for such an ovarian mass is surgical excision, which is usually a curative measure.