Background: Adherence to follow-up visits is crucial for optimizing clinical outcomes in paediatric patients with sickle cell disease (SCD). This study aimed to evaluate compliance with follow-up visits among paediatric SCD patients over a five-year period. Methodology: A retrospective analysis was conducted using data from the sickle cell disease clinic of Nnamdi Azikiwe University Teaching Hospital, spanning from January 2015 to December 2019. A total of 271 unique paediatric patients with SCD and 1117 follow-up visits were included. Compliance with follow-up visits, defined as attendance within 100 days of the previous visit, was assessed using Kaplan-Meier curves and Cox regression analysis. Results: During the study period, a total of 1117 follow-up visits were documented among 271 children diagnosed with sickle cell anaemia. The median age of the study cohort was 8 years (IQR: 4 to 12 years). Male patients constituted 57% of the study population. Fifty percent of subjects remained compliant with follow-up visits at 14.2 months (95% CI = 8.9-24.1 months) and this compliance decreased to 40% at 24 months (95% CI = 31-49 months). Males showed a lower likelihood to be compliant to follow-up visits than females (HR: 0.83; CI: 0.56-1.23). Following adjustments, patients who were sicker and young adolescents (10 to 14 years) had an increased likelihood of complying to follow up visits (HR: 1.05; CI: 0.71-1.57 vs HR: 1.15; CI: 0.682-1.940 respectively) but this was not statistically significant. Conclusion: The overall poor compliance rate to follow up visits underscores the urgent need for interventions aimed at improving adherence to follow-up visits in this vulnerable population. Counselling interventions and further exploration of factors influencing compliance are warranted to enhance the management and outcomes of children living with sickle cell anaemia.