We report a case of a patient undergoing esophagectomy for end-stage achalasia, a rare condition associated with potentially catastrophic ventilatory and circulatory complications. The complexity of the case necessitated preoperative planning with the surgical specialist, development of a novel algorithm for airway management, and careful implementation of our plan. Isolation of the lungs from the esophagus presented unique challenges due to not only the anatomic derangements that are the hallmark of this disease process but also an unusual anatomic finding seen in this patient.