Hobnail hemangioma, also known as targetoid hemosiderotic hemangioma, is an uncommon vascular proliferation that clinically presents as a small solitary red to purple papule or macule, located on the limbs or trunk. Multiple lesions and atypical locations have been described. Histopathologically, it exhibits a biphasic pattern, with dilated vessels in the superficial dermis and angulated vessels in the deeper dermis, with endothelial cells that show a hobnail appearance. There is controversy about the histogenetic origin of hobnail hemangioma, although recent studies support that it is a lymphatic malformation. The investigators report the case of a 41-year-old man with an irregular lesion, red to purple in color, with a maximum diameter of 4 cm, on the scalp. The location and in particular the clinical appearance are uncommon. Immunohistochemical analysis showed negativity for WT1 and focal positivity for D2-40. Clinical-pathologic correlation acquires particular importance in the case of lesions with atypical clinical presentation.