Abstract: In 1996 el Darouti and Abu el Ela described seven Egyptian patients with similar cutaneous lesions and proposed necrolytic acral erythema (NAE) as a distinct entity of the necrolytic erythema family. Since then, NAE has emerged as a cutaneous manifestation of hepatitis C virus infection and taken its place in the literature as a marker for systemic disease. NAE initially presents with burning, pruritic eruptions of circumscribed, erythematous papules with flaccid vesiculation on the acral surfaces universally affecting the dorsum of the feet. The presenting papules of acute NAE evolve over time into confluent, velvety, hyperkeratotic plaques with decreased central erythema but a characteristic dark erythematous rim and adherent scale. Although mostly misdiagnosed as psoriasis or inflammatory dermatitis, NAE can be definitively placed among the necrolytic erythema family as a distinct entity based on clinical and histopathologic characteristics. We report a case of necrolytic acral erythema in a 17-year-old followed by a review of the literature.