Twenty-five cases of double-inlet left ventricle, 23 in atrial situs solitus and 2 with dextroisomerism are described. Twenty had both atrioventricular valves and in 3 a common atrioventricular valve connected to the left ventricle. In rest, the left atrioventricular valve was straddling over the left-sided right ventricle in about 15%. The position of the rudimentary right ventricle varied from superior, anterior and to the right of left ventricle (6 with transposition and 9 with concordant ventriculo arterial connection), to superior anterior and to the left of left ventricle (10 cases, all with transposition). Five cases had stenosis of the left atrioventricular valve and 2 stenosis of the right one. In 11, the clinical presentation was dominated by cyanosis, reduced pulmonary blood flow and right to left shunt. The rest had cyanosis and congestive heart failure. Five patients with right ventricle on the left had complete atrioventricular block, 9 right AQRS orientation and all of them findings of left ventricle hypertrophy. Three cases showed initial Q wave in VI and one left bundle branch block. Cases with right ventricle on the right, had left QRS orientation, left ventricle hypertrophy and 2 complete atrioventricular block. Cross-sectional echoes showed in 13, two atrioventricular valves committed to the left ventricle; in 3 of them the right ventricle was visualized. In 22 cases angiography determined the type of atrioventricular connection. Double inlet left ventricle is one of the most challenging diagnosis in congenital heart disease. Considering its lack of clinical specificity and according to our results, the electrocardiogram, echocardiogram and angiographic findings are of great help for its identification.