We present a rare case of a neonate with an isolated congenital condition of his right ear involving the outer ear, middle ear, eustachian tube, and the facial nerve, with an external opening into the skull that connects to the oropharynx. Taking this bizarre aspect of the exterior lesion and the oropharyngeal communication into account, we consider the condition presented here, which to our knowledge is the first of its kind to have resulted from a vascular disruption. Laryngoscope, 1927-1931, 2018.