Abstract : This case series investigated the outcomes of children who received the maximum tolerated dose of hydroxyurea for sickle cell patients with avascular necrosis of the femoral head (AVNFH) at Maternity and Children Hospital, Makkah, Kingdom of Saudi Arabia (KSA) over one year. This retrospective case series report describes four pediatric patients under the age of 14 with sickle cell disease complicated by AVNFH. We excluded all patients who were non-compliant to medication, missed follow-up, started on another modality of treatment, or did not complete laboratory work-up or imaging. Patients were started on the maximum tolerated dose of hydroxyurea, and the assessment was conducted according to clinical manifestations and radiological findings. Our study found significant clinical and radiological improvement in sickle cell patients with AVNFH who took the maximum tolerated dose of hydroxyurea.