This inherited dominant autosomal condition, associated with a mutation of the tumor suppressor gene HRPT2 in chromosome 1q24–q32 encoding for a protein named parafibromin, is also characterized by the occurrence of parathyroid adenomas or carcinomas, Wilms tumor, renal cysts, renal hamartomas, and benign or malignant uterine tumors [3, 4]. The gene discussed is CDC73; the disease is Wilms tumor.