Corrective operations were attempted in 10 patients with straddling atrioventricular valve (SAVV), and successful palliative operations were performed in another nine patients. SAVV occurred as a part of five different cardiac complexes, and three anatomic types were encountered. The SAVV was a tricuspid valve in eight patients, right atrioventricular valve (in univentricular hearts) in five, and mitral valve in six. Four types of corrective procedures were used: (1) the Rastelli operation, (2) isolated closure of the universally present ventricular septal defect by a patch deviating around the straddling portion of the SAVV, (3) replacement of the SAVV with or without insertion of an extracardiac conduit, and (4) closure of the SAVV plus right atrium-pulmonary artery anastomosis (modified Fontan procedure). All three hospital deaths occurred in patients managed by replacement of SAVV and extracardiac conduit insertion (technique 3). This experience allows preliminary speculation about preferred methods of surgical management.