Background Congenital heart diseases present unique perioperative challenges; when coupled with the altered physiology induced by a phaeochromocytoma, a comprehensive multidisciplinary approach is required. We present the case of a 59-year-old male with cyanotic congenital heart disease and a phaeochromocytoma, exploring the considerations at each stage of his adrenalectomy. Case Presentation A 59-year-old man with left sided phaeochromocytoma was referred to our centre for adrenalectomy and heart transplant waitlisting after developing acute decompensated heart failure, on a background of complex cyanotic congenital heart disease and Eisenmenger’s syndrome. The chronic hypoxia may have contributed to the development of phaeochromocytoma. His perioperative preparation was further complicated by anticoagulation for atrial fibrillation and a recent stroke. Preoperative MDT discussion included consideration of ECMO, alpha-blockade, and the choice of surgical approach in view of his aberrant left sided IVC. Meticulous attention was given to haemodynamic control including on-table trans-oeosophageal echocardiogram. Postoperative vasopressor-dependent vasoplegia necessitated a cardiac pacemaker insertion. The patient was discharged day 15 postoperatively. On follow-up, his exercise capacity and cardiac function had improved to the extent that he no longer required a heart transplant. Conclusion The remarkable long-term outcomes of this case underlines the importance of a considered multidisciplinary approach and at each step of the journey. This case also contributes to the theory that chronic hypoxia increases the risk of phaeochromocytoma development, which in turn can exacerbate underlying cardiac disease.