Apocrine hidrocystoma, altough among the most common of the benign apocrine gland tumors, is still a fairly rare tumor. Although it has been assumed to be a form of retention cyst, it represents a true adnexal neoplasm. Thus, it is better classified as a cystadenoma. Since it is a benign, well circumscribed tumor, it should be treated as all benign cysts. A patient is reported with a giant apocrine hidrocystoma located behind the superior rim of the helix of the left ear.