We report a case of monochorial-monoamniotic twin pregnancy, with diagnosis of an atrioventricular defect, complete truncus arteriosus, pulmonary artery atresia and doubtful anomalous venous drainage in one of the twins. There are no descriptions of similar cases in the literature. After detailed ultrasound follow-up, fetal maturation and elective cesarean section were performed at 32 weeks. The two neonates had low birth weight but Apgar tests and umbilical arteriovenous pH parameters were normal in both. The first newborn twin was diagnosed with congenital heart disease and showed unfavorable postnatal development, dying at 16 days old. Outcome in the second twin was favorable.