Stroke, stroke-like illness and cerebrovascular disease present to the paediatrician in a variety of ways and timely emergency management remains a considerable challenge. In the absence of comprehensive evidence-based guidelines, this article covers the clinical diagnosis, appropriate investigation and management of some of the commoner stroke syndromes presenting in childhood. Anaemia, hypertension and hyperhomocysteinaemia are modifiable risk factors which should be excluded; iron or folate supplementation may be required. Early magnetic resonance imaging with fat-saturated T1 images of the neck to exclude dissection, angiography and venography are often diagnostic. Previously unrecognized cardiac disease is rarely revealed by echocardiography. Randomized controlled trials in adults suggest that intravenous or subcutaneous heparin improve outcome in venous sinus (sinovenous) thrombosis. Anticoagulation may prevent early recurrent embolism after carotid or vertebral dissection. Revascularization often reduces the frequency of transient ischaemic attacks in moyamoya (terminal internal carotid occlusion or severe stenosis with collaterals). Thrombolysis is currently contraindicated outside a 3-h time window which precludes the use of tissue plasminogen activator in most children. The development of clinical networks will allow rapid dissemination of good practice in the management of childhood stroke as the evidence base accumulates.