ABSTRACT Introduction Evidence indicates an increase in the prevalence of enuresis in individuals with sickle cell disease. The present study aims to evaluate the prevalence and impact of enuresis on quality of life in individuals with sickle cell disease. Materials and Methods This cross-sectional study evaluated individuals with sickle cell disease followed at a reference clinic, using a questionnaire designed to evaluate the age of complete toilet training, the presence of enuresis and lower urinary tract, and the impact on quality of life of these individuals. Results Fifty children presenting SCD (52% females, mean age ten years) were included in the study. Of those, 34% (17/50) presented as HbSC, 56% with HbSS (28/50), 2% Sα-thalassemia (1/5) and 8% the type of SCD was not determined. The prevalence of enuresis was 42% (21/50), affecting 75% of subjects at five years and about 15% of adolescents at 15 years of age. Enuresis was classified as monosymptomatic in 33.3% (7/21) and nonmonosymptomatic in 66.6% (14/21) of the cases, being primary in all subjects. Nocturia was identified in 24% (12/50), urgency in 20% (10/50) and daytime incontinence 10% (5/50) of the individuals. Enuresis had a significant impact on the quality of life of 67% of the individuals. Conclusion Enuresis was highly prevalent among children with SCD, and continues to be prevalent throughout early adulthood, being more common in males. Primary nonmonosymptomatic enuresis was the most common type, and 2/3 of the study population had a low quality of life.