Background: Immune checkpoint inhibitors are changing the landscape of oncologic treatment as they are significantly improving treatment of multiple malignancies. Nivolumab, anti PD-1 antibody, is FDA-approved for treatment of melanoma, non-small cell lung cancer and kidney cancer and can cause a spectrum of autoimmune reaction. The adverse effect can occur at any organ system in the body, but most commonly affects colon, lung, liver or endocrine system. Although rare, kidneys also can be involved. Case presentation: A 70 year old male with clear cell kidney cancer was admitted with acute kidney injury while he was receiving nivolumab treatment. A kidney biopsy revealed diffuse tubular injury and immune complex mediated glomerulonephritis. Electron microscopic exam of the specimen demonstrated hump-like subepithelial deposits. Nivolumab was discontinued and the patient was started on high dose steroid. After 5 months of systemic corticosteroid and hemodialysis, patient’s kidney function improved to his baseline. In spite of the prolonged treatment interruption, immunosuppressive therapy did not compromise the anticancer effect of nivolumab. Conclusion: Immune-related adverse effect in kidney can cause autoimmune glomerulonephritis as well as tubulo-interstitial injury. In previous literature, immune-related nephritis generally responded well to systemic corticosteroid. Based our experience, prolonged course of high dose of steroid and hemodialysis may be required to achieve adequate treatment effect.