We present a patient with a relatively rare condition: Charcot joint of the shoulder, with a rare complication, the first known example of combined neurovascular compression in this location. A 49-year-old man presented with neuropathic arthropathy of the shoulder caused by syringomyelia from a Chiari I malformation, leading to compression of both the brachial plexus and the axillary vein by mass effect from the synovitis. The brachial plexopathy resolved with surgical decompression and synovectomy, and the syringomyelia stabilized after Chiari decompression. A large acromioclavicular joint synovial cyst developed as a late complication, which was treated nonoperatively. Understanding neuropathic arthropathy can explain the spectrum of interrelated typical and atypical features in this case over long-term follow-up. Clin. Anat. 26:1017–1023, 2013. © 2012 Wiley Periodicals, Inc.