OBJECTIVE: To study the clinical characteristics of Streptococcus pneumonia-associated hemolytic uremic syndrome (SP-HUS) in children. METHOD: Clinical and laboratory data of a pediatric case of SP-HUS were retrospectively analyzed and the key points of diagnosis and therapy were reviewed. RESULT: An 18-month old girl was admitted with chief complaint of fever and cough for 5 days combined with mild labored breath. Breath sound was found weakened in right lung with lower lobe dullness on percussion. Laboratory tests revealed: WBC 3.7×10(9)/L, Hb 83 g/L, PLT 11×10(9)/L, C-reactive protein (CRP) > 180 mg/L. Morphological study of the RBCs showed marked anisocytosis and schistocytosis. Urinalysis showed 42.66 RBCs per high-power field, occult blood (+++), proteinura (++++). Streptococcus pneumoniae was isolated from blood, pleural fluid and sputum. Serotyping with simplified chessboard system was 3. The direct Coombs test was positive. Serum complement levels (C3 and C4) were depressed at 0.699 g/L, 0.064 g/L, respectively. Chest X-ray showed pleural effusion and infection of the right hemothorax. The computerized tomographic scan of the chest revealed pneumatoceles in the right lower lobe. The diagnosis on admission we considered was SP-HUS. Intravenous antibiotic therapy (vancomycin + cefoperazone/sulbactam) was administered. The renal replacement theraphy was administered to maintain electrolyte and fluid balances and adequate nutrition. Transfusions of washed red blood cells were administered to correct the anemia. One month after admission the patient was good with recovery. Liver and renal function recovered and the pneumonia was resolving, anemia and platelets were corrected. The direct Coombs test turned to be negative. Serum complement levels (C3 and C4) were normal. After 3-month follow-up, no clinical anomalies were detected. CONCLUSION: SP-HUS should be suspected when the following occurs in the context of pneumococcal infections: microangiopathic hemolytic anemia, thrombocytopenia, acute renal failure and a positive Coombs test result. Serotype 3 of SP was associated with HUS.