Fibrous dysplasia was relatively rare disease and firstly described by Weil (1922) and has been well known from 1972 by Lichtenstein and Jaffe. It's peculiarity is an involvement of one, several or many bones of the skeletal system characterized by fibrous connective tissue and prematuration, especially in the case in which skeletal implication is severe and has appeared early in life, abnormal skin pigmentation and premature sexual development may also be present as extraskeletal disorder. Recently we are experienced a rare case of polyostotic fibrous dysplasia which involved skull, facial bone, ribs, scapula, vertebrae, iliac bone and especially complete obstruction of both external ear canal following severe conductive type of hearing loss.