Eagle's syndrome, an uncommon sequela of an elongated styloid process, can manifest itself as a sensation of a foreign body in the throat and a retrogoniac or anterolateral neck pain often referred to the TMJ and the ear. When treating patients affected by temporomandibular disorders (TMD), complaining of atypical orofacial pain, and with a concomitant radiographic finding of a bilaterally elongated styloid, the possible coexistence of Eagle's syndrome should always be considered. The aim of this review is to present an overview of the epidemiology, pathophysiology, diagnosis, and therapy for Eagle's syndrome and to help the clinician in differential diagnosis with other conditions that can provide similar signs and symptoms.