To our knowledge, the coexistence of Warthin tumor and ipsilateral aural atresia has not been reported before. This situation may affect facial nerve structure and present a surgically challenging situation during superficial parotidectomy. A 68-year-old woman with right aural atresia was operated on due to a parotid mass below the atretic ear. Computed tomography and intraoperative findings demonstrated unusual properties of the facial nerve: (i) Extratemporal beginning was more superiorly located, (ii) Facial trunk was relatively longer and more vertical, (iii) Buccal, marginal mandibular, and cervical branching from the unique ring structure of the facial nerve. All facial nerve branches were preserved after careful dissection and the lesion was completely excised. Final histopathologic diagnosis was Warthin tumor. In order to preserve the facial nerve during parotid surgery and to avoid morbidity, it is necessary for surgeons to be familiar with different types of extratemporal facial nerve anatomy especially in cases of additional anomalies such as aural atresia.Keywords: Anatomy, aural atresia, facial nerve, parotidectomy, variation, Warthin tumor.