Study design: Report of a rare case of an elderly patient with late onset of Arnold Chiari malformation type I with associated syringomyelia that was successfully treated with foramen magnum decompression. Objective: To report this rare case along with a literature review. Setting: Gifu, Japan. Methods: A 69-year-old woman with a 4-year history of dull pain in her right arm was referred to the clinic. After physical and radiographical examinations, she was diagnosed with Arnold Chiari malformation type I with associated syringomyelia. A foramen magnum decompression by the removal of the outer layer of the dura mater was performed. Results: At 2 years postoperatively, MRI revealed a decrease in the size of the syringomyelia. Her symptoms had also remarkably improved. Conclusions: A rare case of symptomatic Arnold Chiari malformation type I with associated syringomyelia in an elderly woman was successfully treated with foramen magnum decompression by the removal of the outer layer of the dura mater.Spinal Cord (2005) 43, 249-251.