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Macrodactyly is one of the rarest congenital malformations of the hand. Barsky (1967) found only 64 cases of congenital macrodactyly after a careful review of literature of the past 140 years. Since then a few more reports have appeared in the literature (Bassat et al. 1966, Martinova and Kubacek 1966, Rachnaegel 1967, Tsuge 1967, Tuli et al. 1969, Thorne et al. 1968, Khanna et al. 1975). The present case report is presented because of its rarity and certain unusual clinical features.