Aplasia cutis congenita (ACC) is a rare disorder, which is defined by the localized, or less commonly widespread absence of skin involving the epidermis, dermis and subcutaneous tissue with an incidence of 1/10.000 newborns. The underlying bone and dura mater can be also affected, and muscle and bone involvement occur in approximately 20 to 30% of the cases. Aplasia cutis congenita most often occurs in isolated form, but it has also been reported as part of a heterogeneous group of syndromes. Conservative treatment, surgery, or a combination of these has been described as possible treatment options. In the neonatal period, conservative treatment is more appropriate as a management strategy. We present a rare case of a 2-month old female patient with isolated aplasia cutis congenita, localized on the vertex of the scalp, treated conservatively since birth with good results.