Idiopathic pulmonary arterial hypertension is a rare condition, frequently complicated by pulmonary arteries’ aneurysm. Aggressive medical therapy is often unsatisfactory and lung transplantation remains the only option. We report a unique case of severe idiopathic pulmonary arterial hypertension complicated by a giant pulmonary aneurism, massive pulmonary valve regurgitation, and right ventricle dysfunction. The patient was, as our first choice, listed for heart–lung transplantation and remained in emergency list for more than 7 months. Unfortunately, due to further clinical deterioration and the unavailability of a heart–lung bloc, plan B was mandatory. The patient underwent a combined procedure including: double lung transplant, pulmonary artery plasty, and sutureless pulmonary valve prosthesis with open deployment (first‐in‐man use in such scenario). Postoperative outcome was uneventful. Our thought is that double lung transplantation and conventional combined pulmonary artery/valve surgery should be considered as the first option avoiding excessive waiting times and potential further clinical deterioration.