Also known medically as congenital anterior ear fistula, ear fistula is one of the more common congenital anomalies, and the condition is usually due to a genetic cause with a well-defined hereditary factor. Congenital preauricular fistulas are usually found at birth in newborns with a fistula in front of, behind or in the ear canal. Congenital preauricular fistula is a deformity that occurs during embryonic development, usually due to an underdevelopment of the first or second parotid arch and the parotid fissure. It is usually unilateral, but some patients may have fistulas on both sides of the ear. Patients with congenital preauricular fistulas can be observed without special treatment if infection never occurs, but if localized infection occurs, surgical excision is needed to prevent recurrent infection.