Congenital hand anomalies traverse a vast spectrum from minor deformities to the absent upper limb. It is characterized by patterns of repeated motifs, regulated growth and programmed segmentation. The initiation of limb bud is poorly understood and limb forming potential is present along the entire length of lateral mesoderm. Three major signaling centers interact with each other to control growth and patterning of the limb are apical ectodermal ridge (AER), the zone of polarizing activity (ZPA) and wingless type (Wnt) signaling center. Morphological Development includes musculoskeletal, vascular and neural development. Failure of formation can be transverse or longitudinal. Syndactyly, camptodactyly and clinodactyly are the failures of differentiation. Polydactyly is the most common congenital hand anomaly. It can occur spontaneously or can be familial. It can occur on the preaxial (radial) and the postaxial (ulnar) side of the limb or central. Congenital constriction band syndrome is also called the amniotic band syndrome or Streeter’s disease, characterized by constrictions or bands around digits or limbs.