Aim: Congenital heart disease is the most common congenital problem in children. Presentation can vary from asymptomatic accidental finding to severe cardiac decompensation and death. Early recognition has great implications on prognosis. To study age, gender distribution and frequency of congenital heart disease in children referred to echocardiography in a single hospital in Zarqa. Methods: This is a retrospective descriptive study on all patients with the confirmed diagnosis of congenital heart disease referred for echocardiography over a period of two years from October 2005 to October 2007. Patients from day one of life till 14 years were included. Study was conducted in the pediatric cardiology clinic at Prince Hashem Hospital in Zerqa . Results: A total of (173) children were included. There were (95) males (54.9%) and 78 females (45.1%) with a ratio of 1.2:1. Seventy four percent of the children had acyanotic cardiac lesions. Ventricular septal defect followed by atrial septal defect, patent ductus arteriosus, and pulmonary valve stenosis were the most common acyanotic congenital heart lesions. Whereas Tetralogy of Fallot (TOF) followed by transposition of the great arteries were the commonest cyanotic congenital heart lesions. There was a female dominance in PDA and A-V canal lesions heart defects (63% and 68% respectively). While more males had aortic valve defects. Conclusion: Majority of patients with CHD detected have noncyanotic CHD. TOF is the commonest cyanotic lesion and VSD noncyanotic lesion. In order to avoid complications, early detection of congenital heart disease is of utmost importance for proper management. 2D-echo with Doppler examination forms the gold standard for diagnosis.