IgG4-related aortitis is an inflammatory condition of the aorta, characterized by aortic wall thickening and periaortic soft-tissue involvement. Therefore, this condition can mimic an aortic intramural hematoma (IMH), due to similar radiological findings. We hereby report the case of an IgG4-related aortitis misdiagnosed as an IMH, associated with cerebral hemorrhage, possibly due to cerebral vascular system involvement.