Dear Editor,rnDenys-Drash syndrome (DDS) is characterized by the triad of progressive nephropathy,urogenital malformation and Wilms\' tumor.Incomplete variants present with nephropathy with either intersex disorders or Wilms\' tumor.1 Most DDS patients carry WT1 mutations in exon 8 or 9.1 Here,we report an incomplete DDS child who carried a novel WT1 missense mutation in exon 6 and had unique clinical manifestations.