The incidence of natal and/or neonatal teeth is a rare anomaly, which for centuries has been associated with diverse superstitions among many different ethnic groups. Presence of natal tooth is one of the variations observed in the newborn's oral cavity. The etiology for these teeth is still unknown. An epidemiological, etiological, clinical and therapeutical aspect of this developmental disturbance of dentition is reviewed in order to establish guidelines for the dentist, pediatric dentist, pediatrician, and neonatologist to manage this problem with minimal damage to the future teeth and the newborn baby and to prevent various complications occurring due to this anomalous tooth.