OBJECTIVES: Familial Male-Limited Precocious Puberty (FMPP) is a rare autosomal-dominant genetic condition with sexual dimorphism. We aim to summarize the clinical characteristics of FMPP patients and emphasize the use of a therapeutic regimen involving letrozole, spironolactone, and GnRHa, to augment clinician's understanding of the disease, thus enhancing patient care. METHODS: We retrospectively analyzed the clinical data of 10 FMPP patients and conducted follow-up assessments of adult height in six patients. RESULTS: Out of the 10 FMPP cases, five had the CONCLUSIONS: The most prevalent genotype among FMPP patients in this study was the