Introduction: McCune-Albright syndrome (MAS) is a rare and complex genetic disease that affects the skin, skeleton, and endocrine system. The clinical triad described is composed of polyostotic fibrous bone dysplasia, café-au-lait skin pigmentation, and precocious puberty. When it is associated with myxoma, it is called Mazabraud's syndrome. The variable constellation of symptoms arises from a somatic activating mutation of the GNAS gene, which is present in many types of tissues.

Clinical Case: The case of a 6-year-old patient is presented, with a clinical presentation of a bone lesion in the left superciliary region with progressive growth for 10 months, suggestive of MAS. We focus on the course of the disease and neurosurgical treatment with the Neuronavigation technique, all carried out in our institution.

Conclusion: McCune Albright syndrome is an example of the genetic heterogeneity of polyostotic fibrous dysplasia, it is benign and has a low prevalence, with a great impact on quality of life, therefore treatment must be timely and multidisciplinary.