BackgroundAn osteoma is a benign tumor of bone with unknown etiology and is considered rare, mostly restricted to the craniofacial skeleton.Case presentationThis case report describes an uncommon condylar osteoma in a 67 years old white female patient with laterognathism to the left side, limited mouth opening, aesthetic change and pain associated with the right temporomandibular joint (TMJ). The histopathological examination confirmed osteoma. The lesion was surgically excised and immediate reconstruction was carried out using a custom-made total TMJ prosthesis. The patient has been in follow-up for 2 years, with no symptoms.ConclusionsUnilateral total TMJ prosthesis can be considered to replacement of TMJ after osteoma excision with resection of the condyle.