A 46-year-old man, without a history of hypertension, hyperlipidemia, or type 2 diabetes mellitus, suddenly developed right hemiparesis, reduction of superficial sensation on the left side of the body below the neck, and ataxia of the right limbs followed by right nuchal pain. Dysarthria, hoarseness, dysphagia, hiccup, and paralysis of the right side of the soft palate and tongue were also present. This condition was diagnosed as the Babinski-Nageotte syndrome. Cranial magnetic resonance imaging showed a right hemimedullary infarct at the level of the caudal end of the medulla near the cervicomedullary junction (Figure 1). Magnetic resonance angiography and catheter angiography showed multiple tubular stenoses with adjacent fusiform dilatations of the right vertebral artery, otherwise known as the pearl and string sign (Figure 2), which strongly suggested arterial dissection of the right vertebral artery. His neurological symptoms gradually improved and he was able to walk a month after admission.