Epithelioid hemangioendothelioma, also known as histiocytoid hemangioma, is a clinical and histological tumor between angiosarcoma and hemangioma, which can invade soft tissues and organs such as lung, liver and bone. It is most commonly seen in men before the age of 25. Histopathology: Both dilated blood vessels and spindle cells are seen, and cellular pleomorphism and nuclear division are seen. Staining for reticulin shows reticulated fibers around the outside of individual cells and groups of cells. Immunohistochemical examination was positive for factor 8-related antigen and wattle bean phytohemagglutinin. Epithelial membrane antigens and cytokeratin staining are usually negative. Clinical diagnosis The disease is a multifocal vascular tumor that can invade soft tissues and organs such as lung, liver, and bone. Skin lesions are rare and appear as solitary or multiple slowly arising papules or nodules. It occurs in the upper or lower extremities and is most often seen in men before the age of 25. The skin lesions are often associated with bone involvement beneath them, and the spontaneous symptoms range from asymptomatic to severe burning pain. The diagnosis is made on the basis of clinical presentation with multifocal vascular tumors, the presence of skin lesions is rare and can be diagnosed in combination with histopathological examination. Treatment Extensive resection. Chemotherapy or radiotherapy, or a combination of both, may be used for a small number of metastases.