Hemoglobinopathies are a major public health problem in Saudi Arabia. We studied the effect of splenectomy in 16 Saudi Arabian children with compound hemoglobinopathies. Seven patients no longer require regular blood transfusions, and transfusion requirements were decreased by 30 to 60% in the other eight patients. Three patients whose heights and weights were below the 5th percentile before splenectomy reached the 25th percentile 1 year after the surgery. In spite of preoperative pneumococcal vaccination and the penicillin prophylaxis after the surgery, one patient died of Haemophilus influenzae Group B bacteremia, and three others had six episodes of pneumonia.