The authors describe 4 cases of syringomyelia-associated spinal cord arteriovenous malformation (AVM). All cases were managed with embolization of the AVM. Treatments were aimed to stabilize the AVM itself and not directed toward the syrinx. In 3 of the 4 cases the syringomyelia resolved after treatment. Reports concerning AVM as a cause of syringomyelia is very scarce and lacks posttreatment clinical information. In light of the clinical course and imaging findings, the authors propose a theory that venous hypertension in the spinal cord is the trigger for the development of syringomyelia, which may reverse after AVM treatment.