Adrenocortical carcinoma (ACC) with inferior vena cava thrombosis is rare and has a poor prognosis, and the current literature overwhelmingly supports aggressive surgical intervention. This article summarizes the management of a patient with ACC with inferior vena cava thrombosis, and discusses the feasibility of detailed preoperative imaging data and intraoperative ultrasound to assess the superior and inferior boundaries of ACC with inferior vena cava thrombosis, while describing the intraoperative ultrasound-guided surgical planning and procedure for ACC with retrohepatic inferior vena cava tumor thrombus. Furthermore, it also demonstrates that it is feasible to accurately assess the superior and inferior boundaries of ACC with inferior vena cava thrombosis by preoperative multimodal imaging and intraoperative ultrasound, determine the mode of flow blockage during the operation, and obtain radical resection of the tumor.