Key Points
1. Angiofibroma of tuberous sclerosis complex (TSC) may occur on the auricula and external auditory canal (EAC).
2. Treatment options vary according to the clinical presentation of the disease.
3. EAC involvement of TSC related angiofibroma a rare and specific pathology.
4. EAC obstruction may cause conductive hearing loss and recurrent external otitis.
5. Excision of the angiofibroma with adequate meatoplasty is important to achieve a dry, self-cleaning EAC.
Keywords: Tuberous sclerosis complex, Neurocutaneous syndromes, Hamartomas, External ear, Island flap.