Background: Hemoglobinopathy is inherited anemia characterized by abnormal structure of one of the globin chains of the hemoglobin molecule and has been known to be rare in Korea. However, hemoglobinopathy in children has been reported more frequently than in past with the recent advancement of molecular testing. The purpose of this study was to investigate the clinical and laboratory findings, prevalence and complications of Hemoglobinopathy of children, and to develop the diagnostic process of hemoglobinopathy. Methods: Data of pediatric patients diagnosed with hemoglobinopathy from 2002 to February 2014 at Ajou University Hospital were surveyed. We analyzed patients’ characteristics from clinical and laboratory findings retrospectively. Results: Among a total of 10 children diagnosed with Hemoglobinopathy, 9 patients were confirmed hemoglobinopathy by gene analysis. Eight patients had β thalassemia, 1 hemoglobin D disease, and 1 Hemoglobin Koriyama. In most of the children, anemia was found by accident and most of the children did not have symptoms. In the initial blood test, their mean hemoglobin concentration was 10.0g/dL (range: 4.7-11.2 g/dL), mean corrected reticulocyte count was 3.9% (range: 0.9%-12.2%), and mean total bilirubin level was 1.0 mg/dL (range: 0.3-5.8 mg/dL). Conclusion: The analysis of clinical and laboratory features showed that the characteristics of each type of hemoglobinopathy were similar to that previously reported. We recommend considering hemoglobinopathy if pediatric patient presents with hemolytic anemia. A well organized diagnostic approach including molecular genetic analysis is needed for accurate diagnoses and appropriate management of hemoglobinopathy.