Recent advances in the management and understanding of IgG4-related kidney disease (RKD) have emphasized the importance of urgent treatment in IgG4-related tubulointerstitial nephritis. On the other hand, to avoid long term glucocorticoid toxicity, strategies for early withdrawal of steroid or combination of immunosuppressants, such as rituximab, and the minimum dose of steroid have been pursued. However, disease recurrence after reducing or stopping steroid therapy hampers early withdrawal of glucocorticoid maintenance therapy. In addition, knowledge has accumulated in diagnostic approaches including differential diagnosis of anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, idiopathic multicentric Castleman’s disease, and Rosai-Dorfman disease with kidney lesion, which leads to earlier and precise diagnosis of IgG4-RKD. This review summarizes recent progress in the differential diagnosis of IgG4-RKD and related treatment strategies, and recent topics of hypocomplementemia, membranous glomerulonephritis, and IgG4-related pyelitis and periureteral lesion.