Reports of circulating lipids of children with sickle cell disease (SCD) in Nigeria disagree on the question of whether these children are at increased risk of cardiovascular disease (CVD). We therefore analyzed the serum of 40 females and 37 males with SCD, age 5-19 years, and equal numbers of age-matched controls for total cholesterol, HDL-cholesterol, LDL-cholesterol, triglycerides, and homocysteine. Using bioelectrical impedance analysis, we documented a significant reduction in the per cent fat-free mass in the SCD males and increases in the per cent body fat in both the male and female children with SCD. Marked hypocholesterolemia was present in both genders (means, 100-102 mg/dl) and the LDL-cholesterol levels of the male and female subjects with SCD (54 mg/dl) were below the lower limit of the reference range (59-137 mg/dl). Serum triglycerides in the SCD subjects were in the middle of the reference range for children. Although the mean HDL-cholesterol levels of the SCD males (23.1 mg/dl) and females (24.5 mg/dl) were well below the lower limit of the reference range (35-84 mg/dl), respectively, the LDL-cholesterol/HDL-cholesterol ratios of the SCD subjects were not abnormal. The mean serum homocysteine concentrations of the male and female SCD subjects (9.4-9.6 micromol/l) were at the high end of the normal range. Collectively, these results indicate that children with SCD in northern Nigeria are not at increased risk of CVD. However, their marked hypocholesterolemia should be a cause of concern about the overall mortality and general well-being.