Craniofacial fibrous dysplasia is an extremely complex condition causing fractures and deformity in children and adolescents. Although relatively standard procedures are effective in adults, children require aggressive and innovative intervention if severe deformity is to be avoided. Bone grafting is seldom indicated. The management of each patient must be individualized. The expectations of the parents must be prospectively managed and the patient and parent must be prepared for multiple episodes of reconstructive surgery throughout the growing years. Isolated cases of fibrous displasia in maxillomandibular region are rare and can be difficult to differentiate from other benign and malignant bone disorders. The general dental practitioner can be the first to detect such conditions especially when the only affected areas are in maxillo mandibular region so sufficient knowledge on this condition is important for the proper diagnosis, treatment and prevention of further complications. For obtaining the definite diagnosis, treatment and further management of fibrous dysplasia is mandatory to be carried out imaging studies, histological and laboratory tests.