Congenital scalp defects are the most common manifestation of congenital cutaneous defects (Aplasia Cutis congenita ACC). ACC, in turn, is part of a series of malformations introduced by Cordon in 1767. The main characteristic is the presentation of both localized or multiple skin defects at birth. The most commonly seen clinical manifestation (70%) is an isolated defect of the scalp, and sometimes multiple defects of the scalp may be present. The skin defects are mostly non-inflammatory and well-defined. Lesions can be round, oval, linear, or irregularly star-shaped and can vary in depth from 0.5 to 10 cm. Superficial defects involve only the epidermis or superficial dermis, with no hairs on the surface. Deeper defects may involve the deeper dermis or subcutaneous tissue, and in severe cases the skull is exposed, the skull is missing, or the dura mater or meninges are invaded. Defects of the skin that occur early in pregnancy may present after birth as atrophic, vesicular, membranous or vellum-like scars. Immature defects may appear as ulcers. Most defects occur in isolation at the parietal midline of the head, but occasionally they may be seen on the face, trunk, extremities, or symmetrically. Treatment can be analyzed on a case-by-case basis. It can be healed by scar replacement, by local flap repair, etc. Cranial defect repair is possible when conditions permit.