BACKGROUND: Children with sickle cell disease (SCD) have increased stroke risk, identifiable by elevated velocities on transcranial Doppler (TCD). This review assessed the impact of TCD screening on stroke, mortality, quality of life and morbidity in children with SCD. METHODS: A systematic search of MEDLINE, PubMed, Cochrane libraries, and trial registries was conducted from inception to 28th February 2023. Randomised controlled trials (RCTs) and non-randomised studies (NRS) were included. A meta-analysis and narrative synthesis were performed. FINDINGS: Nine studies were included in the review. In one RCT, initiating chronic blood transfusion in children with abnormal TCD velocities reduced stroke risk by 92 %, while no deaths were reported. Pooled results from three NRS indicated TCD screening leads to four fewer strokes per 1000 patients annually. No studies analysing morbidity nor quality of life were identified. INTERPRETATION: TCD screening may decrease the risk of stroke in patients with SCD.