IgG4-related disease (IgG4-RD) is a systemic condition characterized by elevated serum IgG4 levels and, histopathologically, massive infiltration of IgG4-positive lymphocytes and plasma cells, along with storiform fibrosis, leading to enlargement, nodules, or thickening. This study aimed to investigate the frequency of serum IgG4 elevation and latent IgG4-RD in patients undergoing hemodialysis. Measurement of serum IgG4 levels in 227 patients undergoing hemodialysis from four institutions showed that the mean serum IgG4 level was 34 mg/dL, and serum IgG4 elevation was observed in 12 patients (5.3％). Univariate linear regression analyses indicated that male sex, lower serum CH50 and total cholesterol levels, anti-nuclear antibody positivity, absence of hypertension, and smoking history were associated with serum IgG4 elevation. Age- and sex-adjusted analyses extracted the same factors, except for smoking history, as significant. Secondary evaluations for patients with IgG4 elevation revealed that one of the 12 patients was diagnosed with IgG4-related retroperitoneal fibrosis, which was a cause of renal failure. In patients undergoing hemodialysis, serum IgG4 elevation is more common in males, those with smoking history, or immunological abnormalities. A small proportion of patients with serum IgG4 elevation have latent IgG4-RD；thus, it is important to recognize IgG4-RD as a cause of renal failure.