Abstract: Cherubism is a rare autosomal dominant disorder with incomplete penetrance characterized by extensive pathological bone remodeling according to the high activation of osteoclasts in the lesions within the mandible or maxilla due to heterozygous germline mutations in the gene that encodes the adapter protein SH3BP2. This review focuses on the present research of the function of this gene, its affection to the occurrence of cherubism and the relationship with osteoclast.