We present a case of uterine perivascular epithelioid cell tumor (PEComa) coexisting with both pulmonary lymphangioleiomyomatosis and renal angiomyolipoma. The patient was a 42-year-old woman, suffering from massive abnormal uterine bleeding, progressive dyspnea, and arterial hypertension. Her clinical history included diagnoses of bilateral renal angiomyolipoma and pulmonary lymphangioleiomyomatosis. The patient underwent a subtotal hysterectomy and the specimen contained a subserosal tumor with irregular outlines in addition to uterine leiomyomata. The uterine tumor was histologically composed of solid sheets and tonguelike infiltration and lymphangioleiomyomalike structures within the myometrium, formed by epithelioid cells with ovoid to round nuclei and clear to light eosinophilic cytoplasms. The tumor did not display any morphologic sign of malignancy. The tumor cells showed expression of HMB-45 and smooth muscle markers. Ultrastructural analysis revealed abundant cytoplasmic microfilaments and rare small cytoplasmic bodies with a gridlike lining. We conclude that identification of lymphangioleiomyomalike growth pattern may aid in the differential diagnosis of uterine PEComa and suggest using both PEComa and smooth muscle tumor terminology.