Bart’s syndrome is a rare hereditary mechanobullous disorder, first described by Bart et al in 1966. It is characterized by clinical manifestations: aplasia cutis congenita (ACC) over the lower extremities, epidermolysis bullosa (EB), and nail abnormalities. Previous genetic study revealed glycine substitution mutation in type VII collagen gene (COL7A1) indicated that Bart’s syndrome is a clinical variant of dominant dystrophic EB. The patient was a 1-day-old male newborn who presented with well-demarcated absence of skin over both lower extremities and erosive patches with blisters on nostril, lip, and hand since birth. He was born by Cesarean section at the 37th weeks of gestation. There was no family history of genetic disorder. There was no systemic involvement such as pyloric atresia, renal abnormalities, or arthrogryposis. Histopathologic findings from blister on hand showed subepidermal blister formation, and electron microscopy showed separation of below lamina densa layer. We performed mutation analysis of 118 exons of COL7A1. These result disclosed two novel heterogenous frameshift mutations (c.3841_3843delGGTinsTGGGG in exon 31 and c.8109_81014delGGTGAG in exon 109). Therefore, he was diagnosed as Bart’s syndrome with recessive dystrophic EB. Herein, we report a very rare case of Bart' syndrome which is clinical variant of recessive dystrophic EB, and review genetic mutation in this syndrome.