During the embryological development of the ear, certain malformations may occur. Accessory auricle is one of these developmental anomalies, which has a wide range from a small elevation of skin to a large size to resemble an additional auricle, where the latter called polyotia. Here, we present a case of an accessory auricle in a young female adult. The occurence was unilateral; the subject did not have any audiological disorder. We compared our findings with different cases of accessory auricle in the literature. The clinical importance and genetic association are also discussed.