Objective To report a case of Klinefelter syndrome combined with Kartagener syndrome. Design Case report. Setting Private IVF center. Patient(s) A 35-year-old man with Klinefelter syndrome combined with Kartagener syndrome causing primary infertility. Intervention(s) Testicular sperm extraction (TESE) and intracytoplasmic sperm injection (ICSI). Main Outcome Measure(s) Sperm recovery, fertilization, and live birth. Result(s) Ovulation induction of the female partner, recovery of spermatozoa by TESE from the male partner and ICSI of 9 metaphase II oocytes resulted in two fertilized oocytes. The delivery of a healthy boy with normal anatomy and 46,XY karyotype was achieved after the transfer of only one 4-cell grade 1 embryo. Conclusion(s) To our knowledge, this case with nonmosaic Klinefelter syndrome combined with Kartagener's syndrome is unique and demonstrates the revolutionary aspects of assisted reproductive technologies (ART) concerning male factor infertility.