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BACKGROUND: Red blood cell (RBC) autoimmunization is a relatively uncommon cause of anemia in children and presents some differences from those of adults. Due to its frequency, autoimmune hemolytic anemia (AIHA) in childhood has prompted very few studies, and the literature consists mostly of sporadic case histories. The objective of this study was to stress the importance of an appropriate serologic diagnosis in suspected cases. STUDY DESIGN AND METHODS: This report describes the immunohematologic features of 100 patients with AIHA studied in the Immunohaematologic Unit of Blood Bank, “La Sapienza” University of Rome. The patients were diagnosed in the same department from 1983 to 2003. RESULTS: The peak incidence of AIHA was in the first 4 years of life. No sex predominance was noted. Warm AIHA was the most common type of acquired immune hemolytic anemia; it comprised 64 of the 100 patients, whereas 26 patients showed a cold AIHA. Associated AIHA showed a slightly more frequent incidence (54/100) compared to idiopathic forms of AIHA (46/100). CONCLUSIONS: In this study serologic records of 100 children with confirmed AIHA are reported. This series, much larger than any previously reported, is critically reviewed and analyzed to delineate the immunologic features of the disease in childhood. [ABSTRACT FROM AUTHOR]