Angiokeratomas are vascular malformations that usually appear as multiple or solitary cutaneous plaques. Several clinical variants have been described, with the same underlying histopathological lesion. Mucosal involvement, including the oral cavity, is occasionally found either as a component of the systemic variety, called angiokeratoma corporis diffusum, or associated with cutaneous lesions in more locations. Isolated oral involvement seems to be rather infrequent and only five cases have been described in adults in the world literature. We herein report another case of this rare entity affecting a 62-year-old woman in the dorsum at the tip of the tongue. This is the first report including an immunohistochemical study to discard a lymphatic origin of the tumor.