Cherubism is a rare hereditary developmental condition of the jaws and generally inherited as an autosomal dominant trait. It is also known as familial fibrous dysplasia of the jaws, familial multilocular cystic disease and hereditary fibrous dysplasia of the jaws. The gene for cherubism is mapped to chromosome 4p16.3 may lead to pathologic activation of osteoclasts and disruption of jaw morphogenesis. The lesion usually appears between 2 and 5 years shows a predilection for the mandible and causes a bilateral swelling giving rise to a cherubic chubby appearance. The eosinophilic cuffing of blood vessels appears to be specific for cherubism. The diagnosis is based on clinical, radiographic and histopathologic findings. The purpose of this article is to present a rare case of nonfamilial cherubism as there are very few cases reported and to review the literature with its cone beam computed tomography findings.