Porokeratosis is a rare keratinization disorder of the skin characterized by annular plaques with an atrophic center surrounded by a raised keratotic wall that spreads centrifugally. We report a case of porokeratosis of Mibelli with mutilation. A 30-year-old woman presented with atrophic plaques on the index fingers of both hands with a keratotic ridge in some margins of the plaques. There was loss of the distal phalanx of the left index finger. In the right hand, shortening of the right distal phalanx and flexion contracture of the distal interphalangeal joint were noted in the index finger.