Abstract: Objective To explore the characteristics of patients with post-infectious glomerulonephritis in epidermolysis bullosa (EB), and improve the understanding of the disease.Methods The clinical data of one case of post-infectious glomerulonephritis in EB in our hospital were retrospectively analyzed, and the related literatures were reviewed to examine the clinical features and prognosis of this condition.Results A 52-year-old man exhibited EB at birth, abnormal urine test was found due to a cough two months ago, the renal function and complement were normal, and ASO was not high. The skin biopsy showed squamous epithelium edema, looseness and blister formation, and immunofluorescence was all negative. Renal biopsy showed glomerular segmental mild mesangial proliferative lesion. The immunofluorescence C3+ distributed diffusely, and IgA+ segments were granular in the mesangial region. An isolated cluster of electron dense deposits was observed in the mesangial area under the electronic microscopy. The final diagnosis was post-infectious glomerulonephritis in congenital bullous epidermolysis. The patient was in stable condition with small dose of glucocorticoids combined with tripterygium glycoside tablets.Conclusions The occurrence of renal damage in patients with congenital epidermolysis is extremely rare in clinic. As a clinician, it is necessary to strengthen the examination of urine and kidney function in patients with EB, and renal biopsy is necessary to provide the basis for treatment.