Temporomandibular joint herniation (TMJ) can be caused by inflammation, trauma, tumor, or otologic procedures. However, spontaneous TMJ herniation can also occur as a result of a congenital bony defect in the external auditory canal (EAC), known as the patent foramen of Huschke (PFH), and occurs in 0.4% of the population. Herein, the authors present a case of spontaneous TMJ herniation through the PFH with clicking tinnitus. The patient underwent the surgical repair of bony defect in the EAC with placement of titanium mesh, and the symptom disappeared after surgery. They also review the relevant literature regarding this disease and discuss its embryologic development and clinical significance.