Hyper-IgE syndrome (also known as Job or Buckley syndrome) is a hereditary immune deficiency of autosomal dominant inheritance. It is clinically characterized by the occurrence of recurrent staphylococcal, skin abscesses, bacterial and fungal pneumopathies, and a significant increase in immunoglobulin E. Its association with systemic lupus erythematosus has been described, the mechanism of which involves the deposition of immune complexes. We report the case of an 11-year-old girl with hyper IgE syndrome who was admitted to our clinic with severe renal failure, diagnosed as lupus glomerulonephritis on kidney biopsy.