A congenital preauricular fistula is a problem with the development of the ear early in pregnancy that causes a child to be born with a fistula in the skin in front of the ear. This is a relatively minor malformation and no hearing damage occurs. However, it can lead to frequent infections in the soft tissues of the skin in front of the ear. A congenital preauricular fistula, also known as a preauricular cyst or cleft, creates a sinus tract in the subcutaneous area in front of the ear, which usually leads in the direction of the ear canal, with small openings in the skin area in front of the ear and above the ear canal. In atypical cases, the opening appears below the ear canal, closer to the earlobe. The sinus canal can be short or long and tortuous, with extensive branching, and most occur in the front of one ear. This malformation is found at birth in children, however, it is not associated with hearing impairment and is rarely associated with genetic syndromes involving other problems. The main problem with congenital preauricular fistulas is that they can lead to benign cysts or infections, but infections that are not treated promptly can develop into abscesses. When repeated infections occur, surgical removal of the preauricular fistula is recommended to avoid recurrent infections. Preauricular fistulas that never become infected can also be observed to keep the fistula openings clean. Preauricular fistula is a common congenital condition in children and there is no need to be particularly alarmed. It does not affect a child’s hearing, but can be treated surgically if there are repeated infections or if it affects the appearance.