Abstract
Fibrous dysplasia (FD) is a fibro-osseous lesion of the osseous structures of the body. The exact cause is unknown; however, recently, the cause has been reported to be postzygomatic somatic mutation in guanine nucleotide-binding protein, alpha stimulating 1 gene located at chromosome 20q13.2. The three subtypes of FD are monostotic, polyostotic and craniofacial. The term craniofacial FD (CFD) is used to describe FD where the lesions are confined to contiguous bones of the craniofacial skeleton. This report describes the case of CFD of a 20-year-old male patient who had unusual presentation involving right maxilla and frontal bone of the left side of the face. The clinical features, radiological findings and treatment have been discussed.