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Brown-Séquard syndrome (BSS) is an incomplete spinal cord injury caused by damage to one-half of the spinal cord. Most cases of BSS result from penetrating trauma or tumors, and acute cervical disc herniation is a relatively rare cause of BSS. In this case, a 34-year-old man with a sudden onset posterior neck pain and left side motor weakness was admitted to the local spine hospital. Pain and temperature sensation of pain was decreased below the right C4 dermatome. The left arm and leg motor grade was 0. Magnetic resonance imaging (MRI) showed a huge trans-ligamentous herniated disc rupture from the center to the left at the level of C3–4, and anterior cervical discectomy and fusion were performed. After emergency surgery, left arm and leg motor grade recovered to 2, and normal voiding function returned. MRI verified complete removal of the cervical herniated disc. This case describes the approach to rapid diagnosis in a patient with characteristic clinical symptoms of BSS and radiological findings of a herniated cervical disc. Rapid and accurate diagnosis and immediate decompressive surgery increased the possibility of a good surgical outcome, even if the neurologic deficits are grave at the time of admission.