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Introduction A tracheal bronchus is rarely observed, occurring in only 1% of all patients who undergo thoracic surgeries. We rarely encounter lung cancer in a patient with a tracheal bronchus; however, it is essential to know the distinctive perioperative management strategy for patients with a tracheal bronchus. Case presentation We report a 72-year-old man with lung cancer located in the right apical segment supplied by a tracheal bronchus. Annual chest computed tomography performed as follow-up after colon cancer resection showed an enlarging pulmonary nodule with pure ground-glass opacity, which was suspected to be lung adenocarcinoma. The nodule was located in the right apical segment. The apical segment was independently supplied by a single pulmonary artery superior trunk and a tracheal bronchus that branched directly from the trachea at 1.2 cm above the carina. The pulmonary vein branching pattern was uncommon in that the central vein that usually runs through B2 (posterior bronchus) and B3 (anterior bronchus) was missing. The patient underwent video-assisted thoracoscopic apical segmentectomy under one-lung ventilation using a left-sided double-lumen tube. Discussion Anomalous venous return accompanied with tracheal bronchus has been described in some reports. Since pulmonary vein is important during segmentectomy, the surgeon should pay particular attention to the venous return. Conclusion Preoperative three-dimensional graphic imagery helped us accurately identify the anatomical anomaly to enable the successful segmentectomy in a patient with a tracheal bronchus. We review the relevant literature regarding the perioperative management of patients with a tracheal bronchus., Highlights • A tracheal bronchus is rarely observed in patients who undergo thoracic surgeries. • Be aware of anomalous venous return accompanied with tracheal bronchus. • Tracheal cuff of a double lumen tube may obstruct the tracheal bronchus. • Distance from carina to tracheal bronchus is important for successful anesthesia. • We review the perioperative management of patients with a tracheal bronchus.