Objective: We present an extremely rare case of isolated angiokeratoma of the tongue.
Method: Case report and review of related literature.
Results: An 18-year-old, male adolescent presented with a fleshy, intermittently bleeding mass in the posterior third and base of the tongue. The lesion was initially suspected to be a lingual thyroid or haemangioma, but histopathological features were consistent with angiokeratoma. Magnetic resonance imaging revealed that the lesion extended up to the vallecula and involved the lamina propria and superficial tongue musculature. No similar lesions were found elsewhere in the body. No metabolic derangements were identified in the patient or his family. The 2.6 × 1.5 × 0.5 cm mass was excised under general anaesthesia.
Conclusion: We present the 1st case of isolated lingual angiokeratoma in a male, the 4th such case overally, the largest ever documented. The lesion was situated in the posterior third and base of the tongue, a position not previously described.