Sickle cell anemia is a genetic blood disorder affecting mostly Africans, Hispanics, Indians and people of Middle Eastern descent; involving major organs of which the heart is the most fatal and commonest cause of morbidity and mortality. The present study is aims to determine some electrocardiographic parameters of sickle cell anemia children attending the sickle cell clinic at the University of Port Harcourt Teaching Hospital, Nigeria. A total of 118 subjects comprising of 55 sickle cell anemia (HbSS genotype) patients (Group A) and 63 normal controls consisting of 40 subjects (HbAA genotype) Group B and 23 subjects (HbAS genotype) Group C were recruited into the study. Control subjects were matched for weight and sex with sickle cell anemia (HbSS genotype) (Group A) patients. All subjects were aged between 2 and 15years. Height, weight, body mass index, hemoglobin concentration and heart rates were determined and a thorough physical examination conducted to exclude the presence of co-morbidities. Electrocardiographic parameters were subsequently determined using a standard resting 12-lead electrocardiogram. Results were analyzed using analysis of variance; a p value < 0.05 was considered significant. Results obtained showed no significant differences in age, body mass index and heart rate between patients with sickle cell anemia (HbSS genotype) and the control groups. However, control subjects had significantly higher haemoglobin concentration compared to sickle cell anemia (HbSS genotype) patients (p<0.05). Differences were also observed in some electrocardiographic parameters of sickle cell anemia (HbSS genotype) patients as compared to control subjects. For instance, higher percentages of left and right ventricular hypertrophy, ST segment depression, ischemic changes and axis deviation were observed amongst sickle cell anemia (HbSS genotype) patients compared to control subjects. Further, the QTc interval of Group A sickle cell anemia (HbSS genotype) patients was significantly higher than values for Group C (HbAS genotype) control subjects (p<0.05); however, the p axis of Group A sickle cell anemia (HbSS genotype) patients was significantly lower than values for Group C (HbAS genotype) control subjects (p<0.05). Our study is of value and confirms suggestions that routine electrocardiography amongst children with sickle cell anemia can help detect those prone to arrhythmia, ischaemic changes and sudden cardiac death and therefore enhance mortality and morbidity.