IgG4-related kidney disease (IgG4-RKD) is a manifestation of IgG4-related diseases involving the kidneys and is relatively rare in clinical practice. Its main features are elevated serum IgG4 levels and IgG4-positive cells infiltrating renal interstitial, clinically often manifested as acute or chronic renal insufficiency. This article reported a case of definitive diagnosis of IgG4-RKD in parallel with glucocorticoid therapy. Although the patient had recurrent pancreatitis, he was diagnosed with IgG4-RKD by renal biopsy. When the disease is published in the digestive system, it is necessary to pay attention to whether or not to incorporate multiple systemic injuries for early detection and intervention. The clinical manifestations of the patients were weakness of the lower limbs, no other positive symptoms and signs, serological examination showed that the level of IgG4 was significantly increased, renal biopsy prompted renal interstitial inflammatory cell infiltration and fibrosis, and further immunohistochemistry showed a large number of IgG4-positive cells.