XYY syndrome, also named as super male syndrome, is the most common Y chromosome abnormality disease behind Klinefelter syndrome. The clinical phenotypes of XYY syndrome are quite different. The detection rate of XYY syndrome was low in the past, but with the development of prenatal diagnosis technics and the increasing attention paid by couples to the fetuses, the rate of prenatal diagnosis of fetal XYY syndrome is increasing. Currently, after the intrauterine diagnosis of fetal XYY syndrome, a multidisciplinary team is required to evaluate the prognosis of the fetus, and provide detailed and unbiased clinical consultations to the pregnant couples. After the birth of children with XYY syndrome, multidisciplinary team is still needed to carry out rehabilitation intervention and training for some children with XYY syndrome who have behavioral and cognitive problems. Assisted reproductive technology is needed to improve the pregnancy rate of patients with XYY syndrome and avoid adverse pregnancy outcomes. This article focuses on the latest research progresses in genetic background, diagnosis, fetal perinatal outcomes and postnatal survival, clinical phenotypes, evaluation and treatment of children with XYY syndrome, so as to provide clinical reference for childbearing couples who may be pregnant with children with XYY syndrome.