An autosomal disorder due to pathogenic variants in the WT1 gene leading to an increased risk Wilms tumor and genitourinary abnormalities incorporating Denys-Drash syndrome and Frasier syndrome. The disease is Monarch Disease Ontology entry MONDO_0008679 (Wilms tumor 1). Also known as: WT1-related Wilms tumor predisposition, Wilms tumor type 1, Wilms tumor, autosomal dominant, somatic mutation, Wilms tumor, somatic, Wilms tumor, type 1, autosomal dominant, somatic mutation, Wilms tumour type 1.