Congenital annular multiple fibrofolliculomas occurring with deformity of the ear and ventricular septal defect

We describe a 5-year-old girl who had multiple fibrofolliculomas with an unusual annular configuration, present since birth, localized to the mid- back. She had no family history of similar skin lesions. Examination showed a depigmented patch on her left buttock and other congenital anomalies, i.e. deformity of the auricle of the ear and ventricular septal defect. There has been no previous report of congenital multiple fibrofolliculomas occurring with congenital malformations such as deformity of the auricle of the ear and ventricular septal defect. The congenital occurrence and unusual configuration of the lesions in our patient may suggest a naevoid origin for these tumours.