Objective To understand the clinical characteristics and treatment outcome of Streptococcus pneumoniae-associated hemolytic uremic syndrome (SP-HUS) in children. Methods Clinical data of one child with SP-HUS treated by plasma exchange (PE) were analyzed retrospectively, relevant literatures were reviewed and analyzed. Results The male patient, 3 years and 3 months old, was admitted to the hospital due to cough for 10 days, and aggravated fever and chest pain for 3 days, he had the triad of HUS, Streptococcus pneumoniae was found in both alveolar lavage fluid culture and sputum culture, diagnosis of SP-HUS was confirmed, after active anti-infective combined with 3 times of continuous renal replacement therapy (CRRT) and 2 times of PE therapy, patient's condition gradually improved and was discharged from hospital. The literature review found that two cases of SP-HUS reported in China were 1 year old, 6 months old and 3 years old respectively, all originated from pneumonia, after active anti-infective and supportive therapy, they all improved and discharged from hospital. Conclusion For children with Streptococcus pneumoniae infection, more attention should be paid to the prevention of HUS complications, infants and young children are high-risk groups, and comprehensive therapy is the key to successful therapy.