Klinefelter syndrome patients are mostly clinically azoospermic, and before the era of TESE, were unable to father genetically their own offspring. Nonmosaic Klinefelter [NMK] patients have chances of fatherhood once sperm is harvested from the epididymal seminiferous tubules and further injected in the ovarian cytoplasm. We report a case of 36y old NMK patient, treated for 3 months with supportive and hormonal medication achieving a pregnancy after TESE/ICSI. This is the first published case of successful conception in a couple with a nonmosaic Klinefelter father in Egypt using TESE/ICSI. Review of the literature for any advantages of mTESE over TESE and conception demonstrated higher pregnancy rates by TESE although the sperm retrieval rate was similar in both techniques. Conclusion:Nonmosaic Klinefelter patients should no longer be considered sterile.