Аннотация:This study aimed to describe a case of aplasia cutis congenita with epidermolysis bullosa, as only a few reports of Bart's syndrome exist in the medical literature.Aplasia cutis congenita (ACC) is a rare dermatological condition characterized by the absence of skin.It has no proven etiology and hence stills remains a mystery for the dermatologist of the modern world.The most common body part affected is the scalp.ACC with epidermolysis bullosa (Bart's syndrome) is a very rare variant of aplasia cutis.