Normally, first tooth erupts in oral cavity at the age of 6 months. Eruption of tooth at birth or immediately after birth is a rare phenomenon. The teeth if present at birth are called as natal teeth and those which erupt during first 30 days of birth are called as neonatal teeth. Historical background of natal and neonatal teeth traces way back to 59 BC. It is associated with various complications such as difficulty in suckling, risk of aspiration, sublingual ulcerations, injury to mother’s breast. Hence, the etiological factors along with clinical and histological features, differential diagnosis and its management are briefly discussed.