Aplasia cutis congenita (ACC) is a rarely seen disease in which the dermis, subdermal tissue, and extremely rarely, the bone and dura are absent. ACC predominantly occurs sporadically, but recessive and autosomal dominant patterns of inheritance are described as well. Morbidity and mortality vary according to local and systemic complications. The mortality rate has been reported as 21%. A significant number of cases are lost due to infections, electrolyte imbalance and massive hemorrhage. The type of management has been a subject of controversy in the literature. While some authors prefer conservative treatment, others recommend surgical treatment. Here, we present a female newborn with a large full thickness skin and skull defect on the scalp who was born to consanguineous parents by spontaneous vaginal delivery at 36 weeks’ gestation, with a birth weight of 2160 g. This rare entity and the diverse options for its management are discussed in light of the current literature.

Keywords: aplasia kutis konjenita, saçlı deri, kalvaryum, kanama.