The term aplasia cutis is used to describe congenital localized defects of the skin. The most common site is scalp. The preauricular location is a rare form of aplasia cutis. The presented patient in this paper had skin tag and atrophy in preauricular region from after birth. Skin biopsy was performed, but surgical wound site was not healed. The results of precise investigation, deep biopsy and existence of localized skin atrophy guided to aplasia cutis diagnosis. Aplasia cutis could be recommended in every patient with skin atrophy. The treatments include protection, excision and skin graft or flap.