Pentalogy of Cantrell is a rare entity of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. The complete pentalogy and its variants have been described in the literature. We report a 47-day-old girl with an incomplete pentalogy of Cantrell presenting with congenital absence of the sternum, congenital heart defects, and an epigastric hernia. Two even rarer expressions of this disorder, tricuspid atresia type IIc and asymmetric kidneys, were also found. The patient was successfully managed with palliative pulmonary artery banding as preparation for a modified Fontan operation when she is older. We report this rare case, and discuss the pathologic findings and surgical strategy.