Hematoma is a life-threatening complication of anterior surgery in cervical spondylosis patients. Herein, we report a cervical spondylosis patient complicated with Huntington's disease, who developed unexpected neck hematoma after anterior cervical discectomy and fusion (ACDF) surgical treatment. During the debridement, we found no noticeable vessel lesions and concluded that the occurrence of postoperative hematoma might be due to the drainage displacement caused by excessive uncontrolled movements of the neck after the operation. The patient recovered well, and further literature review suggests that chorea secondary to Huntington's disease likely increases mechanical stress on the cervical spine, indicating an internal relationship between degenerative cervical spondylosis and Huntington's disease. Cervical spondylotic patients complicated with Huntington's disease can be treated with surgical intervention but need to be immobilized and under close observation.