To investigate the clinical and genetic characteristics of children with familial male-limited precocious puberty (FMPP) caused by LHCGR gene. Methods: The clinical data and the gene mutation characteristics of 3 children with FMPP were analyzed retrospectively, and the literatures about FMPP were reviewed. Results: All 3 children with FMPP had presented as the enlargement of penis and had presented as the growth and accelerated growth of the pubes in infancy, and who had visited doctors at the age of 4, 6, or 2.8 years old, respectively. During the first visiting the doctor, the testicular volume was 3 ml in both case 1 and case 3, and was 5 ml in case 2. The bone age of 3 children had significantly advanced, which presented 6, 13 and 5.5 years old, respectively. The testosterone levels of 3 children were 8.14nmol/L, 11.32nmol/L, and 10.29nmol/L, respectively, which had elevated abnormally. The gonadotropin-releasing hormone stimulation test showed that the LH peak of 3 children were all <5mIU/ml, which suggested that there was peripheral precocious puberty in the 3 children. Genetic testing revealed that all 3 children carried heterozygous mutations in the LHCGR gene, including case 1 with c.1730 C>T (p.T577I) from his father, case 2 with c.1713 G>A (p.M571I) from his mother, and case 3 with c.1733 A>T (p.A578V) from his mother. The father of case 1 carried c.1730 C>T heterozygous mutation with a history of precocious puberty, but his adult height reached the normal genetic height. The treatment drugs for children with FMPP were limited, case 3 had no satisfactory therapeutic effect and the prediction of his adult height would short. Conclusion: The main clinical characteristics of the children with FMPP caused by LHCGR gene mutation are peripheral precocious puberty beginning in infancy and high testotoxicosis. The peripheral precocious puberty of these children maybe transform into central precocious puberty, which is more difficult to treat. There is an incomplete dominant inheritance of FMPP of the children, so the clinical phenotype will present as large heterogeneity.