Background: Epithelioid hemangioendothelioma (EH) is a rare vascular neoplasm with an unpredictable malignant potential that has been described mainly in soft tissue, liver, and lung. Case Report: We report a case of a primary renal EH in a 54-year-old woman who presented with a 3-month history of abdominal pain. Abdominal ultrasound and computed tomography scan revealed a solitary mass of 4.0 ×3.2 cm in the outer surface of the right kidney. Nephron-sparing surgery was performed. On the basis of the histological and immunohistochemical findings, the diagnosis of EH was made. The patient was free of disease 6 months after surgery. Conclusions: Although EH may have a histologically benign appearance and can be treated using a nephron-sparing approach, this neoplasm is potentially malignant urging the need for a long-term follow-up in these patients.

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