Eagle’s syndrome is a rare clinical condition caused by the elongation of the styloid process or calcification of the stylohyoid ligament, compressing nearby structures and leading to various symptoms. While 4% of the population may have an elongated styloid process, only a fraction of these individuals are symptomatic. Classic symptoms include pharyngeal pain, otalgia, dysphagia, and headaches, which can be exacerbated by head movements and swallowing. However, atypical neurological presentations involving cranial nerves make diagnosis challenging. This chapter presents several case reports demonstrating unusual manifestations of Eagle’s syndrome, such as facial palsy, tongue paresthesia, Horner’s syndrome, spinal accessory nerve involvement, and seizures. These cases emphasize the syndrome’s capacity to mimic other neurological conditions, underscoring the importance of thorough clinical assessment and imaging, particularly CT scans. Diagnosis and treatment often require a multidisciplinary approach, and while conservative management may suffice for some patients, surgical intervention is necessary for others to alleviate symptoms. Ultimately, this chapter highlights the need for heightened awareness of the condition’s atypical presentations to avoid delays in treatment.