McCune-Albright综合征是以多发性骨纤维发育不良、性早熟、皮肤牛奶咖啡斑三联征为主要表现的临床综合征。该病是一种罕见的鸟核苷酸结合蛋白病(G蛋白病)，由Donovan McCune和Fuller Albright在1936年和1937年首次描述。随着对该病认识的加深，越来越多的患儿被明确诊断。早期的诊断与合理治疗在改善患儿终身高，提高患儿生活质量方面尤其重要。目前提倡应用双膦酸盐治疗多发性骨纤维发育不良，但长期的安全性、有效性尚不明确。针对性早熟的治疗方案较多，最优方案亦不明确。该综述对McCune-Albright综合征的发病机制、临床表现、诊断、治疗，尤其是对多发性骨纤维发育不良、性早熟的治疗研究进行了详细阐述。“,”McCune-Albright syndrome is a clinical syndrome characterized by the triad of polyostotic fibrous dysplasia, precocious puberty, and café-au-lait skin macules.McCune-Albright syndrome is a rare guanine nucleotide-binding protein disease(G-protein disease), and was firstly described by Donovan McCune and Fuller Albright in 1936 and 1937.With the deepening of the understanding of this disease, more and more children have been clearly diagnosed.Early diagnosis and appropriate treatment are especially important in improving the final adult height and the quality of life.The application of bisphosphonates in the treatment of polyostotic fibrous dysplasia is currently advocated, but the long-term safety and effectiveness are not clear.There are some therapeutic strategies for precocious puberty, but the optimal one is not clear.This review elaborates on the pathogenesis, clinical manifestations, diagnosis and treatment of McCune-Albright syndrome, especially the treatment of polyostotic fibrous dysplasia and precocious puberty.