Background: Epithelioid angiomyolipoma is a recently recognized pathologic entity. The occurrence of epithelioid angiomyolipoma in thefemale genital tract is rare.
Case: A case of uterine epithelioid angiomyolipoma occurred in a young woman without tuberous sclerosis and underwent an early recurrence with lymph node metastasis.
Conclusion: Uterine epithelioid angiomyolipoma should be considered in young women with a tumor exhibiting high intratumoral linear vascularity and aneurysmal dilation.