We reviewed 127 patients who were operated upon for adult presentation Chiari malformation and made six conclusions: (a) The clinical examination remains crucial in the diagnosis. (b) The surgical anatomy is highly varied. (c) Syrinxes can be missed on preoperative contrast studies. (d By a conservative grading system, we determined that 46%; of the patients improved during long term follow-up. One-quarter deteriorated over the long run in spite of any treatment. (e) The overall results did not differ whether the treatment was plugging of the central canal plus decompression or decompression alone. (f) In patients with progression, plugging of the central canal obtained superior results. A review of the literature shows that the natural history of this complex disease process has not been established. This history is needed to identify the course of what may be several important factors that lead to the pathological condition in this disease.