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Aplasia cutis congenita (ACC) associated with fetus papyraceus is a rare subtype of aplasia cutis categorized as type V in Frieden's classification. It is characterized by stellate lesions in a symmetrical distribution over the trunk and proximal extremities. Conservative treatment is recommended, but there is not a well-defined therapeutic protocol. We report the case of a type V ACC in a preterm male newborn with lesions on the trunk and scalp successfully treated with topical 1% silver sulfadiazine and petrolatum gauze with an excellent evolution. This case associates a severe affectation of the scalp which represents a rare variant of type V ACC. K E Y W O R D S aplasia cutis congenita, treatment ACC, type V ACC 1 | INTRODUCTION Aplasia cutis congenita (ACC) is a rare disorder characterized by congenital absence of all skin layers in a localized or extensive area. 1 ACC is most frequently found as a solitary lesion on the scalp. However, face, trunk, buttocks and extremities might be affected. 1 It may present as an isolated defect, accompanied by other developmental abnormalities or within a syndrome. 2 Frieden classified ACC into nine groups based on the distribution of the affected area, associated anomalies, and mode of inheritance. 3