Angiomyolipoma (AML) is a benign mesenchymal neoplasm that mainly occurs in the kidney either sporadically or in patients with tuberous sclerosis complex (TSC). Extrarenal AML is uncommon. We describe a 39-year-old female with a history of TSC and bilateral multicentric renal AML who presented with a persistent cystic ovarian mass that fluctuated in size during 2 years of ultrasonographic observation before its removal by salpingo-oophorectomy. The 4.5-cm mass was solid and cystic and tan-yellow. Microscopic examination showed an admixture of epithelioid cells, smooth muscle bundles, large thick-walled blood vessels, and mature adipose tissue. The epithelioid cells had abundant eosinophilic cytoplasm and many had bizarre atypical nuclei including multinucleated forms. Mitoses were rare. Typical smooth muscle cells and the epithelioid cells were strongly immunoreactive for HMB-45. To our knowledge, this represents the first report of an AML arising in the ovary. The differential with other oxyphilic tumors of the ovary is discussed.