A 64-year-old man with primary lung cancer (cT1aN0M0) was diagnosed as having partial anomalous pulmonary venous connection (PAPVC) in the same lobe by preoperative chest computed tomography (CT). The anomalous vein originated from left upper lobe pulmonary vein and flowed into the left brachiocephalic vein. Although the patient was asymptomatic, cardiac catheterization revealed that pulmonary-systemic blood flow ratio (Qp/Qs ratio) was 2.0, and his pulmonary arterial pressure was marginally elevated (60/18 mmHg). We performed left upper lobectomy as the definitive treatment for both lung cancer and PAPVC. His pulmonary arterial pressure decreased after lobectomy (33/16 mmHg). He is living well without relapse of lung cancer 56 months after surgery. Although PAPVC is detectable on computed tomography, out of 7 previous reports of PAPVC associated with lung cancer, only 2 cases were diagnosed preoperatively. The presence of PAPVC should be kept in mind before major lung resections.