Junctional epidermolysis bullosa (EB) with renal and respiratory involvement (junctional EB-RR) is a rare variant of EB caused by biallelic mutations in the integrin α3 (ITGA3) gene. It is characterized by congenital nephrotic syndrome and interstitial lung disease and is associated with premature death.1-5 We report a case of junctional EB-RR limited to the skin and mucosa.