RESUMEN
INTRODUCTION: The midline cervical cleft is a rare congenital anomaly of the anterior part of the neck. CASE: We report the case of a 9-year old girl, presenting a congenital midline cervical cleft who underwent corrective surgery at the age of 7 months. Currently, outome is quite satisfactory two years post-operatively; no recurrence has developed. DISCUSSION: We discuss the clinical manifestation of this anomaly, the embryology, the surgical treatment and the clinical course. In its complete form, this rare disorder presents as a subcutaneous midline vertical cervical cord, associated with a cutaneous cleft. The cutaneous cleft consists of an ulceration with an overhanging cutaneous fibromatous protuberance which prolongs downwards to a blind-ended sinus tract. The embryologic mechanism is not formally established, but the majority of the authors agree on the imperfect midline fusion of the paired branchial arch tissue by anomaly of mesodermisation. Surgical treatment is required and must be carried out in the first month of the life in order to avoid neck and mandible functional and morphological disorders. Surgery involves complete excision of the cutaneous ulceration and subjacent fibrous cord followed by site closure using multiples "Z" plasties. Long postoperative monitoring is needed due to the frequency of the recurrence.