Background. Pentalogy of Cantrell is a rare combined malformation presented with 5 signs: thoracoabdominal defect in the anterior abdominal wall, defects in the lower sternum, the anterior diaphragm and the diaphragmatic pericardium, and congenital heart anomalies. The prognosis is poor. The disease may be diagnosed just in the f irst trimester of pregnancy. Its outcomes and treatment policy are determined by the pattern of cardiac defect and the severity of extracardiac abnormalities. Description. We present a case of three-month survival in a baby from a twin with complete pentalogy of Cantrell. His examination revealed a large anterior abdominal wall defect covered with thin shells. Contractions of the ectopic heart were supraumbilically detected. There were maxillofacial deformities as cleft lip and cleft palate. Systemic muscular hypotonia was pronounced. The fact that the baby had a complex heart defect was a contraindication to surgical correction of omphalocele. In this connection, an attempt was made to partially plunge the eventrated organs into the abdomen by therapeutic methods - omphalocele was vertically recorded behind the umbilical cord at birth (Fig. 1, an insert). The infant with stable hemodynamic parameters maintained with an oxygen concentrator was discharged home when he was 3 months old. Conclusion. When a newborn infant has pentalogy of Cantrell, his management tactic and strategy should be based on the stabilization of hemodynamic parameters and on preparation for further surgical treatment using the current principles of treatment and nursing.