Nager syndrome is a rare hereditary syndrome characterized by severe mandibulofacial and pre-axial upper limb anomalies. Patients with Nager syndrome often exhibit syndromic Pierre-Robin sequence secondary to their hypoplastic mandible and require mandibular distraction osteogenesis to prevent dependence on a tracheostomy or gastric tube. This procedure, however, has been associated with temporomandibular joint (TMJ) ankylosis. TMJ ankylosis is a highly debilitating condition that dramatically impairs patients’ function and can result in facial asymmetry in affected children during periods of rapid facial growth. Surgical intervention is warranted in patients who fail physiotherapy; however, there is no gold standard treatment for surgically correcting TMJ ankylosis in patients with Nager syndrome. Herein, we detail the surgical management of TMJ ankylosis and class 2 malocclusion in a patient with Nager syndrome.