Distinctive genital folliculosebaceous cystic hamartomas in a patient with fibrous dysplasia.

: Folliculosebaceous cystic hamartoma is a benign tumor composed of follicular, sebaceous, and mesenchymal components. Usually, it appears in adulthood as a dome or a pedunculated nodule on the face, especially the nose. Extracephalic lesions are sporadically reported, such as on the upper back, forearm, ear, labia majora, and the nipple. Herein, we report a patient with left maxillary fibrous dysplasia who experienced bilateral labia majora disfigurement after her puberty. She experienced physical distress because of tenderness due to the tangled, stretched hamartomas and psychological distress because of failure to have sexual contacts. The exophytic dark skin-colored fusing polypoid labia majora deformities were excised, and the folliculosebaceous cystic hamartoma was impressed by the pathology. We used an advancing flap for labiaplasty, and the outcome was aesthetically satisfactory. There is no recurrence in the 6-month follow-up period. To our knowledge, this is the first report of a folliculosebaceous cystic hamartoma on the bilateral labia majora in a patient with fibrous dysplasia. This extraordinary presentation is much different from others owing to its unusual appearance and a larger size.