Introduction Tuberous sclerosis complex (TSC) is characterized by hamartomas or angiofibroma in various organs, and numerous papules or nodules that are typically located on the cheeks, nose, and chin specifically. External auditory canal (EAC) involvement of TSC was not reported and is a challenging issue due to external otitis and conductive hear loss besides aesthetical complaint. This report aimed to examine the effectiveness of surgery for the functional and aesthetic management of the TSC for EAC involvement. Patient and Method A 31-year-old male patient with left auricular mass and ear fullness was presented. The patient’s history and family history were compatible with TSC. On physical examination, the total obstruction of EAC was detected due to TSC angiofibroma. Surgical excision and meatoplasty with preauricular fasciocutaneous island flap performed to manage EAC angiofibroma. The pathology specimen was histopathologically diagnosed as angiofibroma. Conclusion Auricula and EAC involvement in TSC related angiofibroma a rare and specific pathology due to the aesthetic and functional feature of the ear. Surgical excision for this clinical entity provides satisfactory management even if it was not routinely recommended for other skin lesions.

Tuberous sclerosis complex (TSC), an autosomal dominant genetic disorder characterized by hamartomas in various organs. Auricula and Meatus Acusticus Externus (MAE) involvement of TSC related angiofibroma is a rare and specific presentation of the disease. A 31-year-old male patient with left auricular mass and ear fullness was presented.