We present a case of a 73-year-old woman who developed a small lesion on her tongue. The nodule was resected and hobnail hemangioma was diagnosed. Hobnail hemangioma is a rare vascular lesion with unusual morphology, including bland cells with hobnail appearance, biphasic grow pattern with superficial dilated vessels and slit-like vessels in the deeper portion of the lesion. The infiltrative pattern of grow may cause misdiagnosis. The differential diagnosis with hemangioendothelioma variants, low grade angiosarcomas and Kaposi sarcoma is of particular concern. The lack of recognition of this uncommon entity may result in excessive and unnecessary treatment.