Persistent left superior vena cava (PLSVC) with absence of right superior vena cava (SVC), also known as isolated PLSVC, is a relatively rare type of congenital body venous malformation. Isolated PLSVC is asymptomatic, however, it will bring clinical difficulties to the implantation of the totally implantable venous access port (TIVAP). We reported a 41 years, male patient with esophageal cancer, who needed neoadjuvant chemoimmunotherapy. Through doppler ultrasonography, computed tomography (CT) and vascular 3D-reconstruction, we found him to be a patient with PLSVC with absence of right SVC before the insertion of TIVAP. Hence, we chose the left approach in which the needle was inserted into the sternocleidomastoid clavicular head lateral notch in left supraclavicular fossa as the puncture point. The depth of the catheter tip from the root of the neck to the puncture point was 21.5 cm and the catheter tip was located at the junction of the PLSVC and the right atrium, at the dilated coronary sinus. The procedure was successful and the patient received expected neoadjuvant chemotherapy combined with immunotherapy after operation, and anticoagulant therapy was performed to prevent thrombosis in coronary sinus and superior vena cava. There was no major catheter-related complication during the period of TIVAP.