Diffuse idiopathic skeletal hyperostosis (DISH) is considered an underdiagnosed and mostly asymptomatic nonprimary osteoarthritis. The etiology of DISH remains unknown and the validated diagnostic criteria are absent. This condition is still recognized radiologically only. Rarely, large projecting anterior osteophytes result in esophageal impingement and distortion leading to dysphagia. We report the case of progressive dysphagia and neck pain due to DISH of the cervical spine in a 70-year-old man, which was surgically removed with excellent postoperative results and complete resolution of symptoms. Imaging studies, surgical findings, and histopathological examinations were used to support the diagnosis. The patient was successfully treated with total excision of the anterior osteophytes with no evidence of recurrence 12 months after surgery. In this report, we also discuss the clinical features and perioperative considerations in combination with a literature review. Our patient illustrates that clinicians should be aware of this rare clinical manifestation as the presenting feature of DISH in cervical spine. Surgical decompression through osteophytectomy is effective for patients who fail conservative treatment.