Surgery is the treatment of choice for compression of the inferior vena cava (IVC) in patients with polycystic liver disease (PCLD). However, hemodynamic instability may develop after induction of anesthesia and during maintenance when compression of the IVC causes a decrease in venous return and may persist until reduction of the volume of the cysts or liver. Perioperative hypotension should be treated promptly by titration of anesthetic drugs, fluid therapy, transfusion, intravenous administration of a vasopressor, and postural modification depending on the etiology. However, regardless of the method(s) chosen, placement in the left lateral decubitus position is crucial for increasing venous return in patients with compression of the IVC. Herein, we report the case of a male patient with PCLD and compression of the IVC who developed refractory hypotension after induction of anesthesia. A dramatic improvement in his hemodynamic status was noted immediately after he was placed in the left lateral decubitus position. The patient’s critical hemodynamic state was then able to be managed until his hepatic cysts were drained, and there were no sequelae.