A 61-year-old female presented with multiple meningeal cysts arising along the whole craniospinal axis, manifesting as trigeminal neuralgia, truncal ataxia, and gradually aggravating headache persisting for 2 years. The patient had suspected Sj äogren syndrome, but no other contributory medical history such as infection, subarachnoid hemorrhage, trauma, malignancies affecting the central nervous system, or habitual drug use. Cerebral magnetic resonance imaging revealed displaced trigeminal root and midbrain, distorted cerebellar hemisphere, and ventriculomegaly, which were relieved by microsurgical resection of the cyst wall. Histological examination confirmed the diagnosis of arachnoid cysts without concomitant meningeal inflammatory reactions. We thought that the multiple meningeal cysts might have originated from undetected meningitis or undetermined underlying mechanism associated with the autoimmune reactions occurring in the arachnoid membrane covering the central nervous system.