<b>Introduction:</b> Eagle’s syndrome (ES) is a rare condition characterized by an elongated styloid process or a calcified stylohyoid ligament. It manifests itself with many symptoms related to anatomical involvement and caused by compression of regional structures, such as temporomandibular disorders, ear pathologies, and facial pain.</br></br> <b>Material and methods:</b> Major online medical databases, including PubMed, Embase, Scopus, and Web of Science were searched to gather all studies regarding symptoms, diagnosis, treatment, coexistence with other diseases and anatomical aspects of the ES.</br></br> <b>Results:</b> A total of 56 studies were included in this systematic review and divided into 5 categories: (1) anatomy and prevalence (n = 19); (2) symptoms (n = 9); (3) diagnosis (n = 8); (4) treatment (n = 20); (5) coexistence with other diseases (n = 5).</br></br> <b>Conclusion:</b> Considering the prevalence of ES and its nonspecific symptoms, it can often lead to misdiagnosis. Hence, all physicians and dentists shall include the ES in the differential diagnosis of cervicofacial and pharyngeal pain.