Pilar sheath acanthoma is a rare, benign follicular hamartoma that frequently presents as an asymptomatic, flesh-colored papule with a central opening. First described in 1978 by Mehregan and Brownstein, these lesions generally appear on the upper lip of elderly patients. We present an interesting case of a pilar sheath acanthoma presenting on the earlobe in a middle-aged male. To the best of our knowledge, this is the first reported case of a pilar sheath acanthoma found in such a unique location.

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