In this article, we present a case of anastomosing hemangioma that shows cystic change. The tumor was a unilocular cystic lesion consisting of 2 distinct layers. The inner layer was composed of a proliferation of capillary-sized blood vessels resembling red pulp of the spleen. The outer layer was composed of stromal cells that resembled Leydig or steroid cell tumor of the ovary. An immunohistochemical examination confirmed endothelial and stromal characteristics of the layers, respectively. An ultrastructural analysis revealed absence of Reinke crystalloids in the stromal cells. We conclude that anastomosing hemangioma may rarely arise from the ovary as a cystic tumor and may be accompanied with luteinization of stromal cells.