Agnathia is one of the first pharyngeal arch deformities referred to as mandibular abnormality. This case report aimed to describe an unusual form of agnathia-otocephaly in a Mehraban ewe-lamb accompanied by other malformations. The scrutiny of the lamb’s face indicated a chain of abnormalities in the head region. Lack of mandible, lips, rima oris, oral cavity, tongue, and teeth were recognized. Eyes and ear lobes were normal, but the base of the pinnae met each other ventral to the atlantooccipital joint and formed a single external acoustic meatus there. The nostrils were normally formed, but the philtrum was not formed due to the lack of lips, especially the upper lip. The hyoid apparatus was normally developed. The laryngopharynx had no connection with the nasopharynx and dead end. Also, the nasal cavities ended blindly because of rinopharyngeal aplasia and no choanal foramen. So, the abnormal pharyngeal region caused a non-functional respiratory system followed by death.
