To report a case of bilateral cervical spinal cord infarction caused by vertebral artery dissection complicated with monoclonal gammopathy of undetermined significance (MGUS). Methods: Clinical data were collected from a patient with spinal cord infarction who was admitted to the neurology department of our hospital in January 2021. Blood biochemical tests, cervical spinal cord MRI, vascular imaging, bone marrow biopsy, and other examinations were performed, and a retrospective analysis was conducted in conjunction with relevant literature. Results: A 68-year-old male patient was admitted with the chief complaint of "right limb weakness." The patient experienced sudden radiating neck pain after making a sharp turn on a motorcycle, followed by gradual onset of right limb paralysis, left limb numbness and weakness, and urinary retention. Neurological examination findings were consistent with spinal cord anterior artery syndrome. Upon admission, the patient had an abnormally elevated D-dimer level, accompanied by intermuscular venous thrombosis in the left lower limb and pulmonary embolism in the right lower lobe of the lung, indicating a hypercoagulable state. Plain MRI and diffusion-weighted imaging of the spinal cord confirmed asymmetric infarction at the C2～C4 levels. Cerebral angiography revealed occlusion of the right vertebral artery at the level of the C3 vertebral body, with multiple stenoses proximal to the occlusion. High-resolution MRI revealed an intramural hematoma in the V2～V4 segments of the right vertebral artery, consistent with vertebral artery dissection. Immunofixation electrophoresis revealed a κ-type IgG monoclonal immunoglobulin band, and bone marrow biopsy showed an increased proportion of monoclonal plasma cells (3.8%), consistent with the diagnosis of MGUS. The patient was treated with rivaroxaban for anticoagulation and rehabilitation therapy, and the hematology department recommended regular follow-up. A literature search identified 60 cases of spinal cord infarction caused by vertebral artery dissection. Analysis of the clinical characteristics of 34 patients with isolated spinal cord infarction revealed that 70.6% were male, 38.2% had triggers such as massage or neck rotation, 94.1% presented with neck pain and limb weakness at onset, 82.4% had unilateral vertebral artery dissection (with unilateral spinal cord infarction accounting for 46.4% and bilateral spinal cord infarction accounting for 42.9% ), and 94.1% received anticoagulation or dual antiplatelet therapy. A literature search identified 61 cases of spinal cord infarction associated with malignancies, of which 33 were hematologic malignancies. Four cases were clearly considered to be caused by a hypercoagulable state associated with the malignancy. One patient with cervical spinal cord infarction considered to be caused by vertebral artery dissection also had M proteinemia and was considered to have concurrent MGUS. Conclusion: When isolated spinal cord infarction presents as an atypical clinical manifestation of vertebral artery dissection, it is necessary to systematically screen for other rare causes of spinal cord infarction. Attention should be paid to the hypercoagulable state caused by malignant proliferative diseases, especially hematologic disorders.