In the last 4⅓ years, 12 episodes of Diplococcus pneumoniae bacteremia or meningitis, or both, were observed in ten children with sickle cell anemia. Seven children were under 2 years of age; seven were boys, and three were girls. The infections were frequently fulminant and two patients died in spite of appropriate therapy. Seven patients required packed erythrocyte transfusions. The infections were caused by eight different serotypes of D pneumoniae. Patients with sickle cell anemia accounted for 7.3% of the children admitted with pneumococcal infections. Pneumococcal infections were present in 2.8% of children admitted with sickle cell anemia.