Cerebral proliferative angiopathy is a rare type of cerebral vascular malformation. It shows distinct angiomorphological features with a clinical presentation that differs to that of classic arteriovenous malformation of the brain. We describe a 15-year-old girl who presented with recurrent headache and limb weakness. A subsequent angiographic diagnosis of cerebral proliferative angiopathy was made. The clinical features, pathophysiology, and treatment of this condition are reviewed.