IgG4-related disease is an emerging clinicopathologic entity. Hypophysitis, diffuse thickening of dura, and enlargement of the trigeminal nerve are well-known intracranial involvements of IgG4-related disease. This report of a case of systemic IgG4-related disease is the first to present neuroimaging of apparent supratentorial meningioma-like lesions and thickening and contrast enhancement of the walls of the intracranial internal carotid arteries. It is important to recognize IgG4-related intracranial pseudotumors so that patients do not undergo unnecessary surgical procedures.