Jeune syndrome (asphyxiating thoracic dystrophy) is a rare inherited disease which is fatal in early childhood in 70% of cases. Severe renal involvement may occur and lead to chronic renal insufficiency in patients who survive respiratory failure. Therefore the opportunity to perform kidney transplantation is quite rare. We report a successful cadaver renal transplantation in a 10-year-old boy with Jeune syndrome type 2.