Over a 7-year period, 110 of 35,000 echocardiographic cases were diagnosed to have total anomalous pulmonary venous connection (TAPVC). Ages ranged from 7 days to 38 years (male 62, female, 48). In 60 cases the diagnosis was confirmed by angiography (n = 47) and/or surgery (n = 50). In 13 cases angiography was not performed; surgery was performed on the basis of echocardiographic diagnosis. Diagnosis of TAPVC was correctly made in all of the 60 confirmed cases. Drainage sites were correctly identified by echocardiography in 58 (96.7%) of these 60 cases. Of the five cases of mixed TAPVC, the second drainage site was missed by echocardiography in two cases. Of the 110 cases the drainage sites were as follows: supracardiac 70, cardiac 30, infracardiac 5, and mixed variety 5. Seventeen cases had Doppler echocardiographic evidence of obstruction along the course of the anomalous vein. The continuous wave Doppler signal for tricuspid regurgitation was present in 14 of 47 catheterized patients, and catheterization-measured peak pulmonary artery systolic pressure correlated well with that derived by Doppler study (r = 0.96, p = 0.001). Additionally, 17 patients had other cardiac anomalies that were correctly diagnosed by echocardiography. Combined two-dimensional and Doppler echocardiography is accurate in the diagnosis of TAPVC, identification of the site of drainage, presence of obstruction, and assessment of pulmonary arterial hypertension and other associated anomalies.