Ameloblastic sarcoma was defined by Pindborg as ""odontogenic tumor composed of benign odontogenic epithelium and having a mesodermal component showing the histological features of a sarcoma."" We recently treated a case of ameloblastic sarcoma of the right maxilla in a 67 year old female and believe that this new and isolated case should be reported, as it is the only way that any reliable knowledge of the diagnosis and treatment can be derived.