Hemangioendotheliomas are vascular tumors which have an intermediate clinical behavior between hemangiomas and angio sarcomas. The epithelioid subtype of hemangioendothelioma, which is rarely seen in bone, has the potential to metastasize and may be confused radiologically with benign or malignant lesions. A metacarpal origin of this tumor is extremely rare. In this article, we present a 42-year-old female case with an hemangioendothelioma in the second metacarpal which was initially managed as a benign lesion before the patient was referred to our center. The potential malignant nature of epithelioid hemangioendothelioma has been ill-defined. We recommend excision with a wide surgical margin. Amputation may be necessary to perform excision in bones such as the metacarpals or phalanges.