Ganglion cysts arising from the temporomandibular joint are rare entities that often present with swelling and minimal to no pain in the preauricular region. To the authors' knowledge, a temporomandibular joint ganglion cyst occurring with acute facial nerve palsy and intracranial extension has never been reported. The patient in the current case initially underwent treatment for Bell palsy and then draining of the cyst at an outside hospital with no relief of symptoms. Repeat MR imaging showed an increase in the size of the cystic, enhancing, middle fossa lesion measuring 4 cm. Resection of the lesion was undertaken using a middle fossa approach. After a satisfactory surgical decompression, the patient demonstrated a significant recovery in her facial palsy over a 3-month period of time. This case presents new clinical and radiographic findings associated with these lesions.