Segmental spinal myoclonus rarely occurs in association with spinal cord tumor. Only 3 cases have been reported in children so far, mainly concerning astrocytomas of the thoracic spinal cord. We report on a 2-year-old boy suffering from segmental spinal myoclonus involving the upper limbs and harboring a cervical tumor. The clinical and electrophysiological features ruled out a myoclonus of different origin (cortical, subcortical, propriospinal) other than other types of movement disorders. Neuroimaging and histological examinations showed the exceptional presence of a ganglioglioma as the cause of the segmental spinal myoclonus. The clinical and electrophysiological characteristics as well as the possible etiopathogenesis and differential diagnosis are discussed on the basis of the pertinent literature to add some more information about the unusual association between spinal cord tumors and spinal myoclonus.