We report a 75-year-old man with glucocorticoid-responsive normal pressure hydrocephalus (NPH) complicated with tubulointerstitial renal disease, protein-losing gastroenteropathy (PLG) and elevated serum IgG4, which might be a possible subset of IgG4-related disease (IgG4-RD). Although either PLG or NPH, especially in combination, has rarely been reported in IgG4-RD, the glucocorticoid-responsive nature of every abnormality observed in the patient supports the diagnosis of IgG4-RD. Of course, pathological confirmation is essential to fulfill the recently raised diagnostic criteria, however, such invasive procedure might not always be indicated in high-risk patients. Our report illustrates that IgG4-RD might be considered as one of the underlying causes and/or the aggravating factors of NPH before shunt operation.