A 38-year-old man presented with a rare case of syringomyelia associated with Paget disease of the skull. Syringosubarachnoid (SS) shunting was performed. We speculate that deformation of the skull secondary to Paget disease caused narrowing of the foramen magnum with progressive impairment of the cerebrospinal fluid circulation, which led to syringomyelia and neurological symptoms. SS shunting is safe, effective, and technically simple, so may be a useful initial treatment for syringomyelia associated with Paget disease of the skull.