Marfan’s syndrome is a systemic disorder of connective tissue typically involving cardiovascular, musculoskeletal and ocular systems. Given the relative rarity of neurovascular complications in Marfan’s syndrome, there is currently little published data on the use of thrombolysis in patients with Marfan’s syndrome and acute ischaemic stroke. Of concern is the possibility of underlying cerebral artery dissection in patients with Marfan’s syndrome presenting with stroke and the risk of haemorrhagic complications with thrombolysis. We report the third known case of a patient with Marfan’s syndrome with an acute ischaemic stroke without evidence of cerebral artery dissection who received thrombolysis successfully with neurological improvement. A 47-year-old woman with a history of Marfan’s syndrome and previous left middle cerebral artery (MCA) territory infarct presented to our emergency department with sudden onset of right facial, arm and leg weakness with a NIHSS score of 15 and clinical examination findings of a right upper motor neurone facial palsy and right hemiparesis. CT brain revealed a dense right MCA sign and no evidence of haemorrhage. She received 0.9mg/kg of alteplase without complications. There was a suspicion for cerebral artery dissection but this was not evident on both CT angiography and MRI angiogram with black blood sequences. She recovered well with a NIHSS score of 1 and mild residual dysphasia. This case demonstrates that thrombolysis may be given safely in a patient with Marfan’s syndrome and acute ischaemic stroke and exclusion of underlying cerebral artery dissection should always be a consideration.