© 2015 North American Society for Pediatric and Adolescent Gynecology. Background: Ovarian teratoma with predominant hemangiomatous component in pediatric patients is very rare and its presentation may mimic that of malignancy. Case: A 2-year-old girl presented with proptosis of the right eye. A right ovarian mass was detected on the physical examination. The right orbital lesion was consistent with venolymphatic-type vascular malformation on magnetic resonance imaging. The abdominal computed tomographic scan revealed a solid ovarian mass that raised a suspicion for malignancy. A 5.5-cm right ovarian solid hemorrhagic mass was resected. Microscopically, the mass was almost exclusively composed of variable-sized blood vessels, with few microscopic mature teratomatous foci. Summary and Conclusion: Hemangiomatous lesion of the ovary may have a presentation mimicking a malignant neoplasm, and may be associated with multiple vascular lesions.