Introduction:A medical condition characterized by the congenital absence of skin, predominantly affecting the lower extremities, blisters on both skin and mucous membranes, and congenital absence and deformities in nails, has been identified within a familial context(1). The inheritance pattern aligns with a fully penetrant, autosomal dominant gene(1).In 1966, Bart introduced Bart syndrome, a condition marked by the simultaneous occurrence of congenital epidermolysis bullosa, localized congenital absence of skin affecting the extremities, and the abnormal shedding or dystrophy of nails(2). This syndrome is fascinating in the medical field due to its more optimistic prognosis when contrasted with other epidermolysis bullosa types(2). This type is known as Aplasia cutis congenital type VI(3).In this study, we have presented a case of a neonate with some disorders that guide us to different diagnoses and syndromes, such as CHIME syndrome and Barth syndrome, that have contributed.