We report a case of neuropathic arthropathy caused by Arnold-Chiari malformation with syringomyelia, with details of the clinical and radiologic aspects. The patient had pain and stiffness in his left upper limb. MRI of the left shoulder revealed neuropathic arthropathy. Upon examination, his left elbow also had the characteristic findings for neuropathic arthropathy. Examination of the spinal cord by MRI confirmed Arnold-Chiari malformation associated with syringomyelia. Neuropathic arthropathy requires evaluation of the spinal cord to assess for occult causal lesions.