A case of first branchial cleft fistula seen by a 4-year-old female was reported. The fistulous tract passed through the parotid gland to the main trunk of the facial nerve, extending from the external cutaneous opening just anterior and below the right angle of the mandibule to the internal opening in the external ear canal. Histopathologically, the tract was lined by stratified squamous epithelium with dermal sebaceous and sweat glands and hair follicles. The upper two-thirds of the tract was surrounded by cartilage, and lymphoid follocles (focal lymphocytic infiltrations) and striated muscle tissues were seen in the lining of the lower one-third. The patient did not show other major anomalies, except the deformed ear drum similar to “Trommelfellschwimmhaut”, a finding repotrted by Oppikofer (1946). Preoperative CT contributed much to locate and identify the tract. Various classifications for the first branchial cleft anomalies proposed by Shaw A et al (1962), Arnot RS (1971), Work WP (1972), and Olsen KD et al (1980), were introduced and compared. A bibliographical review of 143 cases reported in the world literature was made.