Teeth in newborn are called natal teeth and teeth that erupt within one month after birth are called neonatal teeth. Both present as a rare clinical entity. These teeth commonly erupt in the mandibular anterior region. The presence of these teeth may interfere with feeding and present a risk of aspiration and swallowing if they are mobile. Treatment includes extraction. Here we report a case of natal teeth, reviewed the literature regarding the etiology, clinical presentation, associated syndromes and management.