Background <p>Unilateral absence of the pulmonary artery (UAPA) is a rare congenital cardiac malformation. The occurrence of lung cancer associated with UAPA is extremely rare, resulting in limited clinical cases and experience.</p> Case presentation <p>This report presents the case of a 72-year-old female patient with contralateral primary lung cancer complicated by unilateral absence of the left pulmonary artery who underwent surgical treatment. Such patients are particularly prone to intractable hypoxemia during surgery and face elevated risks of postoperative pulmonary hypertension, a situation that poses significant challenges for both surgical intervention and anesthetic management. Through comprehensive preoperative evaluations encompassing cardiopulmonary function and airway status, a thoracoscopic wedge resection of the right lower lobe was performed with bronchial blocker-assisted airway management. A tailored anesthetic protocol was implemented to maintain hemodynamic stability throughout the procedure. Postoperative care featured rigorous monitoring and meticulous nursing management. The surgery was successfully completed, with the patient discharged on postoperative day 5. Histopathological examination confirmed the diagnosis of typical carcinoid.</p> Conclusions <p>For patients with UAPA complicated by contralateral lung cancer, precise isolation of the target lung lobe using a bronchial blocker, combined with comprehensive preoperative evaluation and strict postoperative monitoring, can effectively ensure surgical safety and provide key references for the diagnosis and treatment of similar rare cases.</p> Trial registration <p>This manuscript does not cover a clinical trial. In its place, it furnishes a summarized table of case reports that were previously published.</p>