Jacob disease is the new formation of a pseudo-joint between both of enlarged coronoid process and even zygomatic arch. A 23-year-old female patient with facial asymmetry and limited mouth opening was reported. Computed tomography images showed the classic symptom of Jacob disease with a mushroom-shaped tumor mass from the coronoid process a pseudoarthrosis joint with zygomatic arch. Coronoidectomy and zygomatic arch reduction were planned to operate based on computer-aided design/computer-aided manufacturing. During the actual operation, the excision of coronoid process and reconstruction of zygomatic arch were all navigated by 3-dimensional-printed surgical templates as designed through an intraoral approach. As a result, the enlarged coronoid process was smoothly removed without sequela and mouth opening along with facial symmetry were successfully improved. The authors suggested that computer-aided design/computer-aided manufacturing should be considered as an auxiliary technique to shorten operation time and enhance surgical accuracy.