Children with sickle cell disease may present to doctors anywhere in the world. In developed countries, neonatal screening allows early identification and management of the disease, mostly through daily antibioprophylaxis, immunizations and education of the parents. Stroke prevention relies on the detection of high-risk patients by annual transcranial Doppler ultrasonography from 2 to 16 years of age. Annual check-ups aim to detect early organ deficiencies. The most frequent complications are pain, infections and acute anemia; they may occur in combination. Approximately 10% of children have severe sickle cell disease that may require chronic blood transfusion, hydroxyurea or hematopoietic stem cell transplantation. Comprehensive management programs have dramatically increased survival, and most patients now reach adulthood.