We report a case of a rare heterozygous MYH11:c.3818G>T, p.(Arg1273Leu) missense variant in a patient with multiple brain infarcts early in life, multiple intracranial arterial aneurysms and stenotic lesions, aortic aneurysmal formation, and a familial history of stroke. Here, MYH11 is linked to brain infarction.