Jaw bone is the specialized calcified dense tissue which can be affected by a variety of diseases that often cause it to react in a dynamic fashion. Hereditary diseases affecting the skeleton can be manifested in the jaw; the craniofacial and dental structure. These diseases are heterogeneous in etiology, onset, and severity. Although many of these diseases are individually rare that is affecting less than 1 in 200,000; the total number of people affected is great. Fibro-osseous lesions of bones comprise a different group of pathologic conditions including developmental lesions, reactive or dysplastic lesions and neoplasms. While Fibro-osseous lesions of the craniofacial complex are represented by a variety of disease processes that are characterized by pathologic ossifications and calcifications in association with a hyper cellular fibroblastic marrow element and these lesions shows different microscopic features. This book aimed toward health care professionals in all specialties as a presentable reference manual for recognition, early and definite diagnosis of this diverse group of maxillofacial lesions; diseases of bone manifested in jaw.