We present one of the largest lingual hamartomas of the tongue base to have been reported, along with a review of the current literature and a description of the management of this case, as well as insights into the histopathology of the lesion. A 21-year-old woman presented with a mass on the base of her tongue, extending to the vallecula. The mass was found to be over 4 cm and enhancing on computed tomography. The size, vascularity, and site of the lesion merited its excision using the suprahyoid pharyngotomy approach. Histopathology confirmed the mass to be a vascular hamartoma. In reviewing the literature, we encountered 61 reported cases of lingual hamartomas, which are described with a number of pathological variants and sites of occurrence and with different methods of surgical excision. The size, vascularity, and site of the lesion we found merited a different approach from the conventional transoral approach that was used in all of the previous reports. Also, our study agrees with current world literature that histopathological examination plays an important role in the final diagnosis.