This case highlights the perioperative considerations of a patient with a pheochromocytoma, prior pneumonectomy, and severe mitral valve regurgitation presenting for adrenalectomy. Adrenalectomy for pheochromocytoma carries risks, particularly in patients with cardiopulmonary abnormalities. In this case, we discuss the rationale for proceeding with the adrenalectomy prior to mitral valve intervention. We illustrate the intraoperative surgical and pulmonary challenges in the setting of a prior pneumonectomy. Additionally, we outline the unique considerations during and after pheochromocytoma removal in the setting of mitral regurgitation. Implementation of these considerations is critical for management of a patient with severe cardiopulmonary disease undergoing adrenalectomy.