Cervical misalignment after upper cervical fusion including the occipital bone may cause trismus or dysphagia, because the occipito-atlanto joint is associated with most of the flex and extended motion of the cervical spine. There are no reports of dysphagia and trismus after C1-2 fusion. The purpose of this paper is to demonstrate the potential risk of dysphagia and trismus even after upper cervical short fusion without the occipital bone. The patient was a 69-year-old man with myelopathy caused by os odontoideum and Klippel-Feil syndrome, who developed dysphagia and trismus immediately after C1-2 fusion and C3-6 laminoplasty. Radiographs and CT revealed that his neck posture was extended, but his symptoms still existed a week after surgery. The fixation angle was hyperextended 12 days after the first surgery. His symptoms disappeared immediately after revision surgery. The fixation in the neck-flexed position is thought to be the main cause of the patientʼs post-operative dysphagia and trismus. Dysphagia and trismus may occur even after short upper cervical fusion without the occipital bone or cervical fusion in the neck-extended position. The pre-operative cervical alignment and range of motion of each segment should be thoroughly evaluated.