Background <p>Rarely, the first sign of chronic thromboembolic pulmonary hypertension (CTEPH) is acute embolism, an acute-on-chronic event. These patients are challenging: they need emergent care but are not candidates for immediate definitive treatment with pulmonary endarterectomy (PEA) due to their critical condition.</p> Case presentation <p>We report a patient with massive acute-on-chronic pulmonary thromboembolism leading to cardiogenic shock. We decided on a staged surgical treatment strategy: emergency surgical embolectomy; brindging and stabilization with central veno-arterial extracorporeal membrane oxygenator; and delayed PEA. The patient had full recovery.</p> Conclusions <p>Patients with an undiagnosed CTEPH developing an acute embolism are in a very high risk of facing a complicated recovery and death. A multidisciplinary team effort is warranted. A staged surgical approach with delayed PEA after stabilization seems a reasonable option to manage these rare and complex patients.</p>