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Abstract: Michelin tire syndrome is described in a 22-month-old girl of Indian origin who had generalized folding of the skin, esotropia, epicanthic folds, and delayed speech. Histopathology showed diffuse lipomatous hypertrophy. The skin changes showed partial improvement on follow-up. We report this case because of its rarity. Furthermore, the spontaneous improvement is significant, unlike the persistent course of other congenital nevi.