A patient with chronic recurrent multifocal osteomyelitis is presented for the first time in the dental literature. On the basis of the initial diagnosis of diffuse sclerosing osteomyelitis of the mandible, the clinical course was protracted and refractory to surgical and antibiotic therapies. The literature is reviewed to evaluate the relationship between chronic recurrent multifocal osteomyelitis and diffuse sclerosing osteomyelitis. Distinguishing features between the two entities could not be found. It is recommended that a skeletal survey be performed when the diagnosis of diffuse sclerosing osteomyelitis is established.