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We herein report a rare case of <jats:styled-content style=\"fixed-case\">KFS<\/jats:styled-content> with <jats:styled-content style=\"fixed-case\">CPP<\/jats:styled-content> and review several studies to determine the possible mechanism underlying this condition.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case presentation<\/jats:title><jats:p>An 8\u2010year, 5\u2010month\u2010old male patient was admitted to our hospital because of enlargement of the penis and small testes. Laboratory evaluation revealed high luteinizing hormone and follicle\u2010stimulating hormone levels, a high testosterone level, and the 48,<jats:styled-content style=\"fixed-case\">XXYY<\/jats:styled-content> karyotype. He was treated with triptorelin. One year later, the patient's testosterone level decreased and pubertal arrest occurred.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The literature review in this study showed that the occurrence of hypogonadism combined with <jats:styled-content style=\"fixed-case\">CPP<\/jats:styled-content> is not unique to patients with <jats:styled-content style=\"fixed-case\">KFS<\/jats:styled-content>; it has also been reported in individuals with Turner syndrome, adrenal hypoplasia congenita, and other diseases. Such individuals share common features including partial hypogonadism and a normal hypothalamic\u2010pituitary\u2010gonadal axis. <jats:styled-content style=\"fixed-case\">CPP<\/jats:styled-content> is considered a prelude to hypogonadism, and treatment with a gonadotropin\u2010releasing hormone agonist may prevent gonadal failure.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ped4.12136","type":"journal-article","created":{"date-parts":[[2019,6,25]],"date-time":"2019-06-25T21:28:49Z","timestamp":1561498129000},"page":"127-130","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Central precocious puberty as a prelude to hypogonadism in a patient with Klinefelter syndrome"],"prefix":"10.1002","volume":"3","author":[{"given":"Chunxiu","family":"Gong","sequence":"first","affiliation":[{"name":"Department of Endocrinology Genetics, Metabolism and Adolescent Medicine Beijing Children's Hospital Capital Medical University National 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