This case report describes a 46-year-old man whose first symptom was an attack of acute flank pain, followed by the gradual onset neurological symptoms. We demonstrated a small nidus and serpentine signal-void area in the spinal cord by MRI and diagnosed a glomus type of spinal arteriovenous malformation (AVM). Flank pain is a rare initial finding in an adult with spinal AM In cases with acute flank pain, neurologists should consider spinal AVM as a differential diagnosis. (C) 2004 Elsevier Ltd. All rights reserved.