Vein of Galen Malformation (VGM) is a rare vascular malformation representing less than 1% of vascular intracranial abnormalities. It is a congenital anomaly and frequently detected during the last trimester of gestational age, during neonatal period or early childhood. Clinical manifestations can appear at any age but mainly in the neonatal or infancy stage in the form of heart failure, delayed neuropsychomotor development, hydrocephalus and seizures. First presentation in adult age is very rare. We report a case where a patient remained symptom free until presentation following generalized tonic clonic seizures at the age of 47 years.MRI brain and MR Angiography confirmed the presence of VGM.