Eagle's syndrome, the famous discovery of W. Eagle, is associated with repeated episodes of pain on rotating the neck, dysphagia and referred otalgia due to elongation of styloid process or ossified stylohyoid. Familiarity with Eagle's syndrome shows that its symptoms can be easily confused with other craniomandibular diseases. Since it is a rare clinical entity, its diagnosis is often hard to make, especially in the absence of sound clinical knowledge and good radiographs. Depending on the position of styloid process in relation to underlying anatomical structures, symptoms vary considerably, and in severe cases continuous compression of the carotid artery can lead to stroke and even death. The treatment of choice is mainly styloidectomy; however some conservative treatment is still being practiced. Very few studies have been reported in the literature classifying the elongation and calcification patterns of styloid process. In this review we aim to spread light on the epidemiology, clinical manifestation, diagnosis, and therapy for Eagle's syndrome and also target to help the general practitioner in differential diagnosis with other conditions that can provide similar signs and symptoms.