Atherosclerosis is a significant risk factor for ischemic stroke, and is a frequent cause for extra- and intra-cranial vessels stenosis. Here, we present an unusual case of ischemic stroke associated with intra-cranial vessel stenosis and subarachnoid hemorrhage (SAH) secondary to carotid artery atheroma. A 64-year old female known for hypertension and dyslipidemia presented with a three-day history of three transient episodes (< 30 minutes) of dysarthria and right hand weakness. An initial brain CT scan revealed left frontal SAH. She was admitted to our Stroke Unit for observation and management. CT-angiogram revealed 90% ICAs stenosis bilaterally with several short focal stenotic lesions, involving several left MCA branches. Brain MRI revealed acute infarcts in the left insula, external capsule and inferior frontal gyrus. The clinical picture was attributed to a thromboembolic left MCA ischemic stroke. She was managed with maximum medical therapy, and later underwent successful left carotid endarterectomy. The presence of SAH on our patient’s presenting CT scan lead to an initial radiological diagnosis of RCVS. However, subsequent imaging studies indicated that SAH had occurred in association with a thromboembolic ischemic stroke. Despite its rarity, this clinical association is important to recognize to avoid diagnostic confusion and guide appropriate management.