Nilotinib, one of the tyrosine kinase inhibitors, has been used to treat chronic myeloid leukemia (CML) and Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ ALL). Nilotinib-associated cerebral arterial occlusive disease, which is treated with medicine with/without bypass surgery or stenting, has been sporadically reported to occur. The mechanism of the nilotinib-associated cerebral disease has not been clarified and is still controversial. Here we present the case of a 39-year-old woman with Ph+ ALL treated with nilotinib, which led to symptomatic intracranial arterial stenosis. We performed high-flow bypass surgery and observed the arterial stenotic change in the stenotic portion intraoperatively, whose findings strongly supported the theory of atherosclerosis and seemed to be irreversible.