Extrinsic compression of the left main coronary artery (LMCA) can occur in patients with severe pulmonary hypertension and enlarged pulmonary artery trunk. It has been usually described in the setting of congenital defects such as atrial septal defect, ventricular septal defect, and, more rarely, isolated persistent ductus arteriosus. Functional and structural evaluation of such patients can currently be performed noninvasively with the use of cardiac CT scanning and/or MRI. The optimal management of symptomatic patients remains unknown. We report a case of extrinsic compression of the LMCA in a symptomatic patient with Eisenmenger syndrome who underwent unprotected LMCA stent implantation. We also performed a literature review of the reported cases concerning patients treated with LMCA stent implantation for the management of this condition.