Thus, 46, XX DSD patients who are not mosaic for TSPY are not at a higher risk of cancer.[4] Instances of tumors arising in the gonads of individuals with 46,XX OT-DSD are uncommon.[5, 6, 7, 8, 9, 10] Herein, we present a case of a a male phenotype 46, XX OT-DSD suffering from seminoma, who was successfully treated with surgery combined with neochemotherapy, chemotherapy, and testosterone replacement. This evidence concerns the gene TSPY1 and disorder of sexual differentiation.