Denys-Drash syndrome is characterized by a triad: nephropathy, a 46, XY disorder of sex development, and nephroblastoma with mutations in the gene WT1. A clinical case of a patient with a bilateral metachronous Wilms' tumor, a 46, XY disorder of sex development in the form of a scrotal hypospadias and bilateral abdominal cryptorchidism, without nephropathy with a mutation in 7 exon of gene WT1 is presented in the article. The child underwent left-sided nephrectomy, lower pole right partial nephrectomy, bilateral orchiopexy and two-stage correction of hypospadias. After 7 years from the start of treatment and 3 years after the last procedure, the child’s condition has been assessed as satisfactory. The presented case, according to the analysis of literature, has not been previously described, therefore, it currently remains as “de novo” and requires further observation.

Keywords: Denis-Drash syndrome, nephroblastoma, disorders of sex development, children