Aplasia cutis congenita (ACC) is usually located on the hairy scalp, on the vertex of the head, but can also occur in other locations, such as limbs, trunk. Congenital skin aplasia on the lower limb is very rare disorder. The exact etiopathogenesis is not known, but intrauterine conditions play a role in its development. ACC visually resembles an ulceration, with a smooth pink surface, which in most cases heals spontaneously. Depending on the wound size and whether signs of inflammation are present, the lesion may require local treatment. In the described case, surgical treatment was carried out because of the extreme prematurity of the infant. The outcome was satisfactory, causing no adverse impact on the child's development during the infancy.