Polydactylism is a rare congenital anomaly characterized by the presence of one or more extra digits in cattle. The condition has been reported in several breeds, including Holstein, Simmental, Dexter, Hereford, and Angus, and is generally considered hereditary. Although its exact mode of inheritance remains unclear, evidence suggests involvement of incomplete penetrance, variable expressivity, and polygenic influences. Genes associated with embryonic limb development, particularly those within the Sonic Hedgehog (SHH) signaling pathway, including SHH, LMBR1, GLI3, and HOXD13, are considered important candidates. Clinical manifestations range from minor cosmetic defects to severe locomotor impairment. Polydactylism is of economic and breeding significance because affected animals are typically excluded from breeding programs to prevent transmission of the defect. Despite reports in several cattle breeds worldwide, documented cases in indigenous Indian cattle breeds remain scarce. Advances in genomics may help elucidate the molecular basis of this anomaly and facilitate its management through informed breeding strategies.