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Porokeratosis (PK) is a rare skin disease, of unknown etiology, probably due to a disorder of epidermal keratinization. We describe a 24 year-old male patient, with porokeratosis of Mibelli confirmed histopathologically. A young man of 24, from a non-consanguineous marriage, presented to our department with 4 years history of one hyperkeratotic lesion, symmetric, painless and non-pruritic on the nose. Clinical examination revealed the presence in the left wing of the nose, one annular lesion, measuring about 0.5 cm, the centres was atrophic and surrounded by a raised hyperkeratotic border.