Primary congenital glaucoma is a global problem, which is characterized by increased intraocular pressure and resulted in loss of vision if not timely and appropriately treated. Natal teeth is a rare case which is characterized by the existence of teeth at birth. Its exact etiology is unknown. Herein, a case of a newborn who has a bilateral congenital glaucoma accompanied by natal teeth is presented. The teeth were present in the mandibular incisor region and they were extracted because of the fear of aspiration. The patient also had opacities in his eyes. A diagnosis of congenital glaucoma was made after ophthalmologic examination.