Considering the risk of malignancy in gonads in patients with Swyer syndrome, the data suggest that some pathogenic variants (FTLHL17, DAX-1, DHH R124Q, del at 17q24.3) may be associated with a higher risk and some (STAR, STARD8, MAP3K1) with a lower risk of tumorigenesis. The gene discussed is NR0B1; the disease is 46,XY complete gonadal dysgenesis.