Purpose: To review the literature associated with giant cell tumors in temporomandibular joint and lateral skull base region. Materials and Methods: A review of the literature was performed to record the tumor location, patient’s personal data, and extent of resection, radiotherapy, recurrence, clinical features, and Follow-up. Results: Nine articles were identified and reviewed in the presented study. The most common clinical symptoms in GCTs in the TMJ-SB region were malocclusion and restriction of mouth opening, other symptoms such as persistent headache and hypoacusis also were not rare because of skeletal invasion of the skull base and ears. Skull base GCTs occur most commonly in young adults with an equal sex distribution. The total removal of gaint cell tumors in TMJ without adjuvant radiotherapy is the most effective treatment for localized disease. Conclusion: GCTs of the skull commonly affect young adults, with an equal sex distribution, and are most often centered in temporal bone. Total removal is associated with the lowest recurrence rate and should be the goal of treatment. If total removal cannot be achieved, the combination of subtotal removal and radiation results in a similar recurrence rate.