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B.P.Koirala Institute of Health Sciences, Neurosurgery Unit, Department of Surgery, Dharan, Nepal DOI : 10.5137/1019-5149.JTN.2387-09.2 The McCune-Albright syndrome was described as a syndrome of polyostotic fibrous dysplasia, café au lait skin pigmentation, and autonomous endocrine hyperfunction in 1937. We report a 17-year girl with early menarche and accelerated growth for the past three years. The endocrinological examination showed slight rise of growth hormone with other hormones in normal range. The CT showed an expansive bony lesion over the left parietal area. The bone mass was excised with bone cement cranioplasty performed for the defect. Histology confirmed it was fibrous dysplasia. Although uncommon, this syndrome must be kept in mind in cases with bony abnormalities and extensive endocrinological workup done and treatment given for best results. Keywords : Café au lait, Fibrous dysplasia, McCune-Albright syndrome