Schönlein-Henoch nephritis (SHN) usually presents with micro-haematuria or mild proteinuria but can be associated with heavy proteinuria and nephrotic or acute nephritic syndrome. Although the treatment of SHN is still under debate, it has been suggested that cyclosporin A (CsA) may be beneficial in children with severe SHN, as reported by Ronkainen et al. (Pediatr Nephrol 18:1138-1142, 2003) and Someya et al. (Pediatr Int 46:111-113, 2004). However, histological effects of CsA on severe SHN had not been evaluated in their evaluations. We report the successful use of CsA in a male patient with severe SHN with nephrotic-range proteinuria, who failed to respond to methylprednisolone pulse and azathioprine therapy.