Background: Sickle Cell Disease is the commonest monogenic haemoglobinopathy worldwide. Living with a long-term condition such as sickle cell disease during adolescence constitutes a significant challenge for the key stakeholders due to the combined effects of chronic illness and adolescent development. For adolescents with sickle cell disease to be cared for and supported appropriately and effectively, it is crucial that health professionals have a comprehensive knowledge and understanding of how adolescents experience living with the condition. While there is developing literature about how adolescent's experience sickle cell disease, this body of research has not been critically reviewed and synthesised. Objective: To identify, critically appraise and synthesise primary research exploring adolescents’ experiences of living with sickle cell disease to make recommendations for practice and research. Design: Integrative narrative review. Data sources: A systematic search of 10 electronic databases and key journals was conducted to identify studies from the inception of databases to September 2016. Review method: Inclusion criteria: adolescents with sickle cell disease aged 12–19 years, primary data on adolescents’ own perspectives, and published in English. Data were extracted on study contexts, methodology/design, theoretical constructs, participants, and key findings. Findings from included studies were synthesised using the integrative narrative approach. Additionally, the methodological quality of studies was assessed using the Hawker et al. (2002) appraisal checklist. Results: 683 studies were identified, of which 40 fulfilled the inclusion criteria. Nine broad themes emerged: knowledge and understanding of the condition, symptom experiences, self-management, attitude to treatment, healthcare experiences, social relationships, difference and striving for normality, school experiences, and emotional well-being and coping. Majority of the studies were of moderate quality methodologically. Quality assessment demonstrated a high risk of bias in three studies. Conclusions: Sickle cell disease impacts on multiple facets of an adolescent's life. While there are similarities in the experience of living with sickle cell disease and living with other chronic illnesses, there are essential differences in relationship dynamics and healthcare experience. The adolescents expressed less confidence in generic healthcare providers. The review highlights areas relating to symptom management and health service provision that has been under-researched and need further exploration to understand adolescents’ experiences and their support needs fully. Nursing care and research should focus more on adolescents’ developmental wellbeing, promote peer support network among adolescents with the condition and with adolescents with other chronic illnesses and collaborate with adolescents to ensure service development are developmentally and culturally appropriate.