An unusual presentation of branchial cleft fistula penetrating the submandibular gland

Abstract First branchial cleft anomalies constitute a rare entity with variable clinical presentations and anatomic findings. We describe a 14-month-old girl with a congenital cutaneous fistula running from a cutaneous opening in the left submandibular triangle through the submandibular gland and ending in the pharyngeal cavity. These features suggested type II first branchial cleft fistula with an extremely unusual path. Complete excision resulted in successful treatment without recurrence. Since the first branchial cleft fistula can originate anywhere along the salivary gland and can extend from this area, surgeons should maintain a high index of suspicion for anatomic variants of this rare condition.