BACKGROUND: Temporomandibular joint (TMJ) ankylosis can be accompanied by various degrees of functional and esthetic problems. Adequate mouth opening, occlusal stability, and harmonious facial form are the main goals of treatment for ankylosis. Distraction osteogenesis has proven to be an excellent treatment for lengthening the ramus-condyle unit. However, various timings for distraction have been suggested, and there is no consensus on selection criteria for performing the procedure in stages or simultaneously with other treatments. CASE PRESENTATION: In this case report, concomitant intraoral distraction and gap arthroplasty was planned to treat TMJ ankylosis and associated facial asymmetry. After gap arthroplasty and 23 mm of distraction, the ramus-condyle segment was successfully lengthened and mouth opening range was significantly increased. The resultant interocclusal space was stably maintained with an occlusal splint for 4 months after distraction. Finally, good occlusion was achieved after prosthetic treatment. The remaining mandibular asymmetry was corrected with osseous contouring and augmentation surgery. The mouth-opening range was maintained at 35 mm 24 months after treatment. CONCLUSION: Gap arthroplasty with intraoral distraction as a one-stage treatment and subsequent contouring surgery can be applied to correct ankylosis with moderate malocclusion and facial asymmetry.