Idiopathic granulomatous angiitis of the central nervous system (IGANS) is a rare vasculitis primarily affecting the spinal cord and brain not related to any underlying systemic disease. Clinical manifestations range from simple headache to cerebral vascular accidents secondary to vascular occlusion. The management of a pregnancy complicated by multiple risk factors including underlying IGANS is reported. An expectant, empiric approach was adopted and resulted in excellent maternal and neonatal outcomes. The first report of the management of a pregnancy complicated by IGANS is presented. Underlying maternal IGANS may not necessarily represent an indication for pregnancy termination.