Objective. To evaluate the influence of hip dysfunction on quality of life of pediatric patients with sickle cell disease (SCD). Methods. Clinical demographic, Charnley hip score, Barthel scale and Pediatric Quality of Life Inventory (PedsQL 4.0) questionnaires were used. Results. Seventeen patients were placed into the group “with hip dysfunction,” and 54 into the group “without hip dysfunction.” The group “with hip dysfunction” had a higher average age ( P = .026), weight ( P = .029), and height ( P = .019) than the group “without hip dysfunction.” There was also a higher prevalence of hip trauma ( P = .05) and pain ( P = .00). The study demonstrated that hip dysfunction negatively influenced quality of life in the physical activity ( P = .11) and psychosocial ( P = .003) domains. Conclusion. Hip dysfunction and previous hip trauma negatively influenced quality of life, especially in the physical activity and psychosocial domains.