Objective To explore the value of echocardiography in the diagnosis of the criss-cross heart. Methods The echocardiographic results of 6 patients with the criss-cross heart were reviewed retrospectively. The echocardiographic characteristics were analyzed and compared with the surgery results in 4 operated cases. Results The 6 cases were interpreted as representing a criss-cross heart with solitus atria,D-loop ventricles and concordant atrioventricular connections. The ventriculo-arterial alignments of 5 cases were abnormal including double outlet right ventricle with anterior aorta in 2 and transposition of the great arteries in 3 while 1 patient had concordant connection. The following principal characteristics were tilting the transducer from posterior to anterior could demonstrate the connection of the left-sided left atrium and the right-sided left ventricle through mitral valve at first. The more anterior angulation of the transducer then showed the right-sided right atrium was connected to the left-sided right ventricle through tricuspid imaging displayed the two atrial outflows crossed each other without mixing at atrioventricular valve level.right ventricle often occured. The echocardiographic diagnosis of the criss-cross heart and its associated cardiac abnormalities were confirmed by surgery in 4 cases except 1 persistent left superior vena cava was missed. Conclusions The invisibility of a standard 4-chamber view in any cut was very characteristic in the echocardiographic diagnosis of the criss-cross heart. The definitive appearance was the separate display of the two ventricular inlets and the crossed atrioventricular connections with each atrium emptying into the contralateral ventricle by continuous subxiphoid or apical scanning. The transthoracic echocardiography can diagnose this rare heart disease and associated cardiac abnormalities accurately.