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INTRODUCTION: Epithelioid hemangioendothelioma is a tumor of soft tissues arising from the vascular endothelium. It is considered as a low-grade malignant tumor. Cutaneous involvement is rare and often associated with multi-systemic localizations. CASE REPORT: We report the case of a 34 year-old woman with a 6-month history of a 1.5 cm erythematous-violaceous, soft, painful cutaneous nodule involving the right forearm. An abdominal sonography had been performed 1 month before the onset of the nodule, because of epigastric pain and was normal. Histological and immunohistological examinations led to diagnosis of an epithelioid hemangioendothelioma. Assessment of the extension with radiology, sonography, tomodensitometry and magnetic resonance imaging, revealed nodules of the liver. Treatment consisted in the wide and complete excision of the tumor. There was no evidence of local recurrence after one year follow-up, and the hepatic lesions were stable. DISCUSSION: Epithelioid hemangioendotheliomas belong to the epithelioid vascular tumor spectrum. They have in common the morphologic epithelioid aspect of endothelial tumor cells. Cutaneous localization is rare, and to the best of our knowledge, only 20 cases of epithelioid hemangioendothelioma with skin involvement have been reported in the literature. Epithelioid hemangioendothelioma can be isolated or associated with internal visceral involvement. The detection of skin lesions should lead to a complete assessment of the extension of the disease to detect any internal localization. Because of low-grade malignancy of the tumor, and the integrity of the liver observed one month before the onset of the disease in our patient, epithelioid hemangioendothelioma appears to be a multicentric disease rather than metastatic hepatic localizations of a primitive skin cancer.