In the present case report we describe a rare case of a 32-year-old female patient with a history of blurring of vision since 1 month. There was history of sudden onset of severe headache. This case report highlights the step wise approach to diagnose a case left superior quadrantic hemianopia in a case of right occipital lobe arteriovenous malformation presenting with severe headache and its management by gamma knife radiation surgery leading to promising results.