Introduction. Epithelioid hemangioendothelioma is a tumor of soft tissues aris ing from the vascular endothelium. It is considered as a low-grade malignant tu mor. Cutaneous involvement is rare and often associated with multi-systemic loc alizations. Case report. We report the case of a 34 year-old woman with a 6-mo nth 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. Hist ological and immunohistological examinations led to diagnosis of an epithelioid hemangioendothelioma. Assessment of the extension with radiology, sonography, to modensitometry and magnetic resonance imaging, revealed nodules of the liver. Tr eatment consisted in the wide and complete excision of the tumor. There was no e vidence of local recurrence after one year follow-up, and the hepatic lesions w ere stable. Discussion. Epithelioid hemangioendotheliomas belong to the epitheli oid vascular tumor spectrum. They have in common the morphologic epithelioid asp ect of endothelial tumor cells. Cutaneouslocalizationisrare,andtothebestofourkno wledge, only 20 cases of epithelioid hemangioendothelioma with skin involvement have been reported in the literature. Epithelioid hemangioendotheliomacanbe isol atedorassociatedwithinternal 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 th e integrity of the liver observed one month before the onset of the disease in o ur patient, epithelioid hemangioendothelioma appears to be a multicentric diseas e rather than metastatic hepatic localizations of a primitive skin cancer. Introduction. Epithelioid hemangioendothelioma is a tumor of soft tissues aris ing from the vascular endothelium. It is considered as a low-grade malignant tu mor. Cutaneous involvement is rare and often associated with multi-systemic loc alizations. Case report. We report the case of a 34 year-old woman with a 6-mo nth 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, to modensitometry and magnetic resonance imaging, revealed nodules of the liver. Tr eatment 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 w ere stable. Discussion. Epithelioid hem They have in common the morphologic epithelioid asp ect of endothelial tumor cells. Cutaneous localization of brain, and to treat end of kno wledge, only 20 cases of epithelioid hemangioendothelioma with skin involvement have been reported in the literature. Epithelioid hemangioendotheliomacanbe isol atedorassociated with internal visceral 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 th e integrity of the liver observed one month before the onset of the disease in o ur patient, epithelioid hemangioendothelioma appears to be multicentric diseas e rather than metastatic hepatic localizations of a primitive skin cancer.