BackgroundAnatomic variation may increase the difficulty and risk for anatomic segmentectomy. The preoperative three-dimensional computed tomography bronchography and angiography(3D-CTBA) can provide a detailed model of the segmental structure, and contribute to precise and safe segmentectomy.Case presentationWe report a case with anomalous bronchi and pulmonary vessels in the right upper posterior segment (RS2), under the guidance of 3D-CTBA, anatomic RS2segmentoctomywas performed accurately and safely, the postoperative condition was uneventful.ConclusionsThis rare case highlights the importance of 3D-CTBA to guild accurate segmentectomy with anatomic variation.