In 1983, we had two cases of pediatric patients, which were observed in the Regional Hospital "Victor Lazarte Echegaray" of the Peruvian Institute of Social Security in Trujillo, these cases correspond to the syndrome of aplasia cutis congenita. In the first case, the defect was localized as a of ulcer in the right inferior member, which was affected from the third part distal of the thigh to the back part of the foot. In the second one, the lesion was in the middle line of the hairy skin, and it had the particularity of being associate to others congenital malformations. These cases are mentioned because of their rarity of presentation at a world level as well as their unknown occurrence in our country. Then we comment the clinic, histological characteristics, associate congenital defects, possible etiopathogenic mechanisms, differential diagnostic and its evolution and treatment.