Hormonally inactive adrenocortical carcinoma (ACC) is a rare disease where abdominal discomfort and back pain are common presenting symptoms due to mass effect from a large tumor. Acute kidney injury (AKI) from retroperitoneal tumors has rarely been reported. The most common etiologies include venous thrombosis, ureteral compression, or both. Here, we described a man who presented with AKI from a large retroperitoneal tumor, which was finally diagnosed as a non-functional ACC. The inferior vena cava (IVC) was nearly completely compressed by the large retroperitoneal tumor leading to venous outflow obstruction and AKI. After surgical resection, his urine output increased and renal function recovered. Unfortunately, AKI recurred 2 months later due to recurrence of the tumor. Treatment with a tyrosine kinase inhibitor stabilized his tumor size, and hemodialysis was started. IVC-compression-associated AKI can be the presenting scenario for ACC, a rare but prognostically important aggressive neoplasm.