Introduction: Nevus sebaceous is a benign mass lesion occurring primarily on the face, scalp, and periauricular region. The occurrence of this lesion in the external auditory canal is very rare.
Case description: We present a case of nevus sebaceous of the external auditory canal, which was diagnosed with the help of histopathology and treated successfully by surgical excision.
Discussion: The nevus sebaceous is a benign lesion but harbors malignant potential in it, necessitating excision of the lesion with adequate margins in order to avoid recurrence or malignant transformation.
Conclusion: Nevus sebaceous although rare but can arise in the external ear canal and can be managed successfully if excised completely with adequate margins.
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