Objectives: Enuresis in children with sickle cell disease (SCD) is common. Many risk factors have been postulated but its relation to hyposthenuria is debatable. This study aims to determine the prevalence of enuresis inchildren with SCD in Basrah, Iraq and to examine its relation with hyposthenuria. Methods: A cross-sectional epidemiological study was performed on children with SCD who met the inclusion criteria at the Basrah Center for Hereditary Blood Diseases over the period from the first of December 2020 through May 2021. A questionnaire was used to collect relevant data. Blood samples were tested for hemoglobin genotype, certain blood indices, and serum hemoglobin. Urine was tested for albumin and creatinine, and the specific gravity was measured using urine dipsticks. The relationships between enuresis and various sociodemographic and clinical variables were assessed. Binary logistic regression analysis was done to examine the independent risk factors of enuresis. Results: Out of 200 eligible children, 161 were studied after exclusion of 39 based on the exclusion criteria, yielding an 80.5% response rate, 60.9% of them were males. The mean age of the participants was 10.9 ± 2.9 years. Enuresis was reported in 50 (31.1%) patients. The independent risk factors for enuresis were; family history of enuresis (OR, 5.94; 95% CI, 2.54-13.89; P<0.001), hyposthenuria (OR, 3.76, 95% CI, 1.25-11.30; P= 0.018), and sleep disorders (OR, 2.90; 95% CI, 0.19-7.06; P= 0.019). Conclusion: Enuresis is common among children with SCD. Hyposthenuria was significantly associated with enuresis. Family history of enuresis, and sleep disorders were also found to be significantly related to enuresis. Keywords: Enuresis, sickle cell disease, children, prevalence, hyposthenuria