Introduction. Aplasia cutis congenita (ACC) is a very rare, congenital benign pathology. The incidence of the disease is unknown, so far around 500 cases have been reported. The prognosis for ACC is usually good. ACC was described by Cordon in 1767, being manifested as a solitary, scarring lesion, involving various layers of the skin and sometimes the bone. The lesions may occur anywhere on the body, but in 70% it is located on the scalp. ACC can occur as an isolated defect or can be associated with other anomalies. An ACC classification by Frieden I.J. consists of 9 basic groups, with the absence or presence of associated malformations. Case presentation. We present the case of a 10-month-old male patient, whose mother requested a dermatologist consultation for a congenital scarring alopecia lesion on the scalp. The child was born from a healthy pregnancy with no perinatal or hereditary history of pathology. The lesion manifested as a solitary, round-ovular, non-inflammatory, pink-pale well-demarcated, easily folded site of scarring alopecia at the vertex, measuring 2x3 cm in diameter. The hair around the lesion had a much more rapid growth, being darker and coarser than the rest of the hair on the scalp. Specialist consultation both at birth and in hospital (neurologist, ophthalmologist, pediatrician, cardiologist) did not reveal any pathology and/or deformities. The distinct character of the case was recurrent contagious impetigo. Conc lus ions . The presented case is included in the rarest congenital skin diseases, which can associate various syndromes, presenting both clinical and scientific interest.