We report the clinical case of a 15-year-old boy who presented with progressive throbbing headache, associated with the demonstrated arteriovenous malformation (AVM), initially without any neurological symptoms and no familial history of headache. This chapter aims to provide a comprehensive overview of headaches attributed to AVMs, including their epidemiology, clinical presentation, pathophysiology, diagnosis, and treatment. By addressing these aspects, we aim to raise awareness and enhance the management of this complex condition.