Cherubism is a congenital childhood disease of autosomal dominant inheritance. This disease is characterized by painless bilateral enlargement of the jaws, in which bone is replaced with fibrous tissue. The condition has sui generis clinical, radiographic and histological features, of which the clinician should be aware for a better differential diagnosis in the presence of a fibro-osseous lesion affecting the bones of the maxillomandibular complex. The purpose of present paper was to review the literature and to report the most important aspects of cherubism in order to facilitate the study of this disease. Literature was reviewed about cherubism, emphasizing the relevant clinicoradiographic features and treatment. Literature was selected through a search of PubMed and Scielo electronic databases. The keywords used for search were adolescent, cherubism, cherubism/physiopathology, cherubism/treatment, cherubism/radiography. A manual search of the reference lists of the identified articles and the authors' article files and recent reviews was conducted to identify additional publications. Those studies that described new features about cherubism were included in this review. In total 44 literature sources were obtained and reviewed. Studies that described new features about cherubism physiopathology, diagnostics and treatment were reviewed. Despite the exceptions, cherubism is a clinically well-characterized disease. In cases of a suspicion of cherubism, radiographic examination is essential since the clinical presentation, the location and distribution of the lesions may define the diagnosis. Histopathological examination is complementary. Nowadays, genetic tests should be used for final diagnosis of cherubism.Postgraduation Program in Oral Biopathology, São José dos Campos Dental School, São Paulo State University - UNESP, São José dos Campos, São Paulo Brazil.Department of Biosciences and Oral Diagnosis, São José dos Campos Dental School, São Paulo State University - UNESP, São José dos Campos, São Paulo Brazil.Postgraduation Program in Oral Biopathology, São José dos Campos Dental School, São Paulo State University - UNESP, São José dos Campos, São Paulo Brazil.Department of Biosciences and Oral Diagnosis, São José dos Campos Dental School, São Paulo State University - UNESP, São José dos Campos, São Paulo Brazil.