BACKGROUND Children with sickle cell disease (SCD) are at potential risk of iron overload from chronic transfusion and probable iron deficiency due to accelerated growth. However, only few studies on the iron status of children with SCD in Nigeria are available. METHODS A cross-sectional study compared the iron status of 109 children with sickle cell disease with 109 age and sex-matched haemoglobin AA controls at the University of Ilorin Teaching Hospital. Parameters assayed were serum iron, ferritin, transferrin, and haemoglobin (Hb) concentrations. Considering the appropriate reference values for age and sex, these parameters were used to classify the children into high, normal, or low iron status. RESULTS The median (interquartile range) serum ferritin level of 180.00 (237.50)ng/ml for the SCD subjects was significantly higher than 70.00 (120.00)ng/ml observed among controls, but the mean Hb and median serum transferrin levels were significantly lower in the subjects compared with the corresponding values in the controls, each showing statistical significance (p<0.05). The median serum iron levels did not differ significantly between the SCD (112µg/dl) and non-SCD (128µg/dl), p=0.309. A high proportion of subjects had low HB status (96.3%) compared with controls (56.9%), p=0.001. A significantly higher proportion of subjects (78%) had high ferritin status compared with the controls (48.3%; p <0.001). Ten (9.1%) SCD children had low serum iron status compared to 28 (25.7%) HbAA controls, p=0.002. Thirty-four (31.2) subjects had low transferrin status which was significantly higher than the corresponding number of controls (8;7.3%; p<0.001). CONCLUSIONS The children with SCD in the index study were iron-sufficient.