Dent’s disease type 1: A differential for steroid-resistant nephrotic syndrome

Abstract Dent’s disease is an X-linked recessive renal tubular disease with varied clinical presentations. It may present with isolated proteinuria simulating nephrotic syndrome. Here, we report a child who presented with steroid-resistant nephrotic syndrome (SRNS) and was subsequently diagnosed with Dent’s disease type 1 by genetic testing and progressed to chronic kidney disease stage 3. There was a positive family history with the mother being the carrier and the uncle having isolated sub nephrotic proteinuria. We report this case to highlight Dent’s disease as the differential of SRNS and to substantiate the lack of genotype–phenotype correlation.