We present a case of a neonate with Shone complex in addition to the underdevelopment of the right ventricle associated with dysplasia and stenosis of the tricuspid valve. Because of lack of effective surgical options, the patient was transplanted at the age of 8 days. A gradient was noted in the postoperative period across the inferior vena cava anastomosis. At reoperation, a prominent myxomatous Eustachian valve was resected. We believe the large Eustachian valve was likely responsible for the right-sided pathology.