To analyze the karyotype distribution, pregnancy outcomes, and postnatal phenotype of fetuses with sex chromosome aneuploidy (SCA). Methods: A total of 11063 pregnant women who underwent amniocentesis for prenatal diagnosis in the hospital from July 2011 to April 2022 were enrolled in this study. The prenatal diagnostic indications of these women included the advanced age, the high risk of the fetal chromosomal abnormalities through serological screening, the high risk of the fetal free DNA by non-invasive prenatal testing (NIPT), the abnormal results of the prenatal ultrasound examination and the history of adverse pregnancy and childbirth. The chromosome karyotype of the fetal cells in amniotic fluid and the prenatal diagnostic indications of the women were analyzed, then the pregnancy outcomes and the postnatal phenotype of the fetuses with SCA were followed up. Results: A total of 76 fetuses with SCA were found, with a detection rate of 0.7%. Among the SCA, 47, XXY accounted for 21.0%, 47, XXX accounted for 14.5%, 47, XYY accounted for 9.2%, 45, X accounted for 7.9%, 48, XXYY accounted for 1.3% and chimerism accounted for 46.0%. Among 76 women had the fetuses with SCA, 2 cases were lost to follow-up, and 17 (22.4%) cases continued to conceive. There were 57 (75.0%) women with the terminated pregnancy, including 16 cases with the fetal 47, XXY, 5 cases with the fetal 47, XXX, 5 cases with the fetal 47, XYY, 6 cases with the fetal 45, X, 1 case with the fetal 48, XXYY and 24 cases with the fetal chimerism, and the terminated pregnancy rates of which were 100%, 45.5%, 71.4%, 100%, 100%, and 68.6%, respectively. The most common indications of the prenatal diagnosis for the high-risk fetuses were the abnormal results of serological screening (31.6%) and NIPT of fetal free DNA (29.0%). The follow-up of 17 fetuses with SCA after birth were conducted. 1 infant with 47, XXX was followed up to 4 years and 4 months old and its delayed language and intellectual development accompanied by epileptic seizures was found. 1 infant with 47, XYY was followed up to 1 year and 2 months old and its concealed penis was found. The other 15 infants with SCA showed no the abnormalities in the physical development, the language development, the cognition, the physiological structure and the behavior during the follow up. Conclusion: The detection rate of SCA of the fetuses was 0.7%. The common prenatal diagnostic indications of the pregnant women with the fetal SCA are the high-risk results of the serological screening during mid pregnancy and NIPT. The combination of the serological screening and NIPT techniques combined with the amniotic fluid chromosome karyotype analysis can help to detect the fetuses with SCA. The women with the fetal 47, XXY and 45, X have the higher rate of termination pregnancy. For the fetuses with 47, XXX or SCA chimera, most of them have the normal postnatal phenotypes after birth.