Charcot arthropathy is a progressive, chronic and degenerative destruction of one or several joints caused by a central or peripheral neurological disorder. Approximately 25 % of the patients with syringomyelia develop this arthropathy located in the upper limb in 80 % of the cases. An early etiological diagnosis is essential to begin the treatment of the underlying neurological disorder. Afterwards, a conservative treatment of the arthropathy is preferred. We report the story of a patient with an arthropathy of the left shoulder due to Arnold-Chiari's malformation of type I with syringomyelia.