ObjectiveTo investigate the clinical features of Hirayama disease (HD). MethodsWe analyzed the clinical manifestations and assistant examination results of one patient with HD diagnosed in November, 2010. In addition, we reviewed the related literatures. ResultsThe patient was a young man, and the main symptoms were bilateral hand weakness with muscular atrophy. The muscle bundles trembled when he stretched his fingers. The electromyography showed neurogenic damage of both the thenar muscles and the first interosseous muscles. Brain magnetic resonance imaging (MRI) showed normal; cervical spine flexion MRI showed thinning cervical spinal cord with spinal canal narrowing at C5-C7 plane. In addition, epidural fat gap widened at C4-T1 plane, and enhanced scan showed cord-like changes, patchy strengthening and blood flow void shadow. ConclusionWhen asymmetric distal upper limb muscle weakness with muscle atrophy appears in adolescents, HD should be considered. The combination of neuroelectrophysiological examination and cervical spine flexion MRI scan is helpful for diagnosis. Wearing cervical collar may slow disease progression.