Odontogenic keratocyst is an epithelial developmental odontogenic cyst most commonly occurring in the jaws. It comprises approximately 11% of all cysts of the jaws. It has an aggressive behavior including high rates of recurrence, rapid growth, and extension into adjacent tissues. Odontogenic keratocyst is commonly found in the mandible with a predilection for angle and ascending ramus of the mandible. We document a case of odontogenic keratocyst that is unusually originated from the temporomandibular joint and we review the existing literature concerning odontogenic keratocyst. As far we know this is the first case of the odontogenic keratocyst originating from the temporomandibular joint in the English-written literature.