Purpose: Cyanotic congenital heart disease (CCHD) induces secondary erythrocytosis and CCHD is thought to be one of the risk factors for cholelithiasis, but the frequency of cholelithiasis and/or gallstones in patients with CCHD is unknown. Materials and Methods: A hundred and two patients with CHD were divided into 4 groups: group A 22 CCHD without reparative surgery or with palliative surgery; group B 35 CCHD after repair who were eliminated cyanosis; group C 17 acyanotic CHD without repair; and group D 28 acyanotic CHD after repair. Gallstones were studied by abdominal ultrasonography or CT in each patient. For analysis of risk factors, patient records regarding cyanotic period, family history and past abdominal surgery were reviewed and blood pressures, lipid profiles, blood sugar and liver enzyme were evaluated. Results: Cholecystectomy due to cholelithiasis was underwent in 7 of 279 CCHD patients (2.5%). In group A, 7 patients (31%) underwent cholecystectomy, and 3 patients (18%) have gallstones. In group B, 11 patients (37%) had gallstones. In group C, nobody had gallstones, and in group D 2 patients (7%) had gallstones. SPO2 was significantly lower and red blood cell, hemoglobin, hematocrit, HbA1C and total bilirubin levels were significantly higher in group A (p 19.8, 95%CI 1.30 300.1). Conclusions: The incidence of cholelithiasis and gallstones is significantly high in CCHD regardless of repair. The most important factor related to gallstones was having CCHD even after desaturation was improved by surgical repair.