First described in 1955, Van Buchem disease is an infrequently occurring hereditary sclerosing bone dysplasia. It is more properly called hyperostosis corticalis generalisata. The most striking feature is an unusual enlargement of the mandible with a normal dental occlusion. Skull base, spine, and pelvic bone involvement is also seen. The defect appears to be an increase in cortical bone thickness or sclerosis. The condition first appears around puberty in the autosomal recessive type and in early childhood with the autosomal dominant type. Reported here is a family with Van Buchem disease, in which surgical recontouring of the mandible was performed for one of the members. The surgery was performed by a combined intraoral/extraoral technique with good aesthetic results and minimal morbidity. A differential diagnosis and workup is also presented.