Congenital anomalies of the upper extremity are noted in approximately 2 per 1000 live births. By modified classification of Oberg, all anomalies are placed within one of three groups: malformations, deformations, and dysplasias. Treatment of these patients is multidisciplinary. Polydactyly (presence of extra digits or duplication of digital parts) is the most frequently observed congenital hand anomaly; it is easily detected, and it causes cosmetic and functional impairment. It is roughly classified as radial, which is most common, central, and ulnar. Treatment options are different and they range from simple excision to complex microvascular reconstruction. Synostosis, Kirner’s deformity, brachydactyly, triphalangeal thumb, clinodactyly, camptodactyly, macrodactyly, thumb hypoplasia, congenital constriction band syndrome, and congenital trigger thumb occur as the most common hand anomalies in pediatric population with various clinical presentations and requiring different treatment options.