Background: Sickle Cell Disease (SCD) is a genetic disorder in which red blood cells undergo episodes of sickling following hypoxemia. Episodes of ischaemic crisis in children are predicted to accelerate the decline in pulmonary function. There are few published reports on lung function assessments in children with sickle-cell disease in sub-Saharan Africa. This study aimed to investigate the association between patterns of spirometry assessment in children with SCD in Ghana. Method: A case-control study involving 115 children aged 6-16 years with SCD and 115 non-SCD controls was carried out at the Komfo Anokye Teaching Hospital. Demographic, socio-economic, and clinical information were collected. Spirometry was performed for 130 cases and controls at enrolment. Results: Children with SCD had lower lung volumes and lower anthropometric measurements compared to the controls: forced expiratory volume in 1 second (FEV 1), cases vs controls, 1.40L vs 1.80L, (p<0.001), forced vital capacity (FVC), cases vs controls,1.70L vs 2.10L, (p<0.001), body mass index(BMI), cases vs controls,16.0 kg/m 2 vs 17.2 kg/m 2, (p= 0.002), height, cases vs controls, 135cm vs 143cm, (p<0.001) weight, cases vs controls, 31kg vs 38kg, (p<0.001). Restrictive abnormality on spirometry was predominant, 43% (n=49) in cases compared to 25% (n=29) in controls. Obstructive abnormality was uncommon. We observed an association between older age and restrictive pattern on spirometry (OR 1.53, 95% CI 1.23-21.91, p <0.001) as well as BMI and restrictive pattern on spirometry (OR 0.63, CI 0.48-0.81, p<0.001) Conclusion: This study has demonstrated a predominance of low anthropometric measurements and restrictive abnormality on spirometry in children with SCD in Ghana. Restrictive pulmonary function abnormality is associated with older age and lower BMI.