First branchial cleft fistula presenting with external opening on earlobe

First branchial cleft fistula is a rare congenital malformation of the head and neck with an incidence of less than 10% of all branchial cleft defects. We herein report a 15-year-old girl who had a cystic mass in the postauricular region with an external opening on the posterior face of the earlobe. Surgical exploration revealed that a second sinus tract was passing through the conchal cartilage without going beyond the skin of the external acoustic meatus. The mass and the tract were excised along with the opening on the earlobe as well as the skin island surrounding the opening. The case was treated surgically with success .The significance of our case was the location of external opening on the earlobe.