Neuropathic arthropathy (NA), is a progressive degenerative arthropathy associated with an underlying central or peripheral neurologic disorder. Neuropathic arthropathy has been observed in a variety of conditions including syringomyelia, diabetes mellitus and peripheral nerve disorders. The shoulder and elbow joints are the most frequently affected while the hand joints are quite rarely seen in the cases of NA caused by syringomyelia. In this article, we report the case of neuropathic arthropathy in a patient with Arnold-Chiari malformation type I and syringomyelia probably caused by a cerebellopontine angle (CPA) arachnoid cyst. Case : A 39-years old man came with slight weakness in both upper and lower extremities and spastic-related deformities in both hands accompanied with a history of headache. Cranial nerves examinations showed rotatoar nystagmus. Plain radiograph examinations of both hands demonstrated destructive osteoarthritic changes and multiple ossified intraarticular bodies consistent with intra-articular osteophytes. He was investigated with magnetic resonance imaging (MRI) of the brain and the cervicothoracic spine and they revealed arachnoid cyst at cerebelopontine cisterna causing the cerebellar tonsils downward displacement across foramen magnum (Arnold-Chiari type I malformation) with communican hydrochepalus and a syringomyelic cavity extending from C2 to Th5 vertebral levels. Bed rest and lowering-intracaranial pressure therapy with acetazolamide was given to the patient. Conclusion: Development of neuropathic atrophy following the diagnosis and surgery of syringomyelia should be considered especially in destructive cases without evident pain complaint and the underlying neurological problem should be surmounted.