The goal of this paper has been to count and describe congenital heart disease associated with double conus. We studied 196 pathologic specimens from to the Pathologic Collection of the Department of Embryology of the National Institute of Cardiology "Ignacio Chávez" of México. We found 3% of double conus in classic tricuspid atresia with concordant ventriculoarterial connection, 53% with double outlet right ventricle, 16% with discordant ventriculoarterial connection, 91% with double outlet single ventricle, 8% with one outlet right ventricle with pulmonary atresia, 20% with one outlet left ventricle and 25% with one outlet single ventricle. The morphological data of the associated anomalies were gathered in an informatized sheet designed according the segmental and sequential approach to diagnose congenital heart disease. From our results we conclude that double conus cannot be considered pathognomonic of any congenital cardiopathy, although they appear associated more frequently with truncoconal anomalies and single ventricle. It is frequent the association between double conus and stenosis of one of them, specially the subpulmonary one. We found great association between left juxtaposition of the atrial appendages and double conus.