Pulmonary Alveolar Microlithiasis (PAM) is a rare autosomal recessive end stage lung condition characterized by widespread alveolar deposition of calcium microliths. It is worth emphasis india has reported 80 cases out of 1022 cases worldwide but there is no report on lung transplant and and anaesthetic management in this category of patient. This report present the anaesthetic challenge in management of first indian patient with the diagnosis of PAM, who underwent bilateral lung transplantation. Bilateral lung transplantation is one of the most challenging surgeries that require the care of a cardiothoracic anesthesiologist. Utilization of extracorporeal circulation has allowed a safer performance of this procedure in patients with severe cardiopulmonary compromise. Intraoperative management is a pivotal part of the patient's care, as it contributes to the patient's overall outcome.