Immunoglobulin (Ig) G4-related disease is a newly recognized fibroinflammatory condition involving every organ system such as pancreas, kidney, lung, lymph nodes, meninges, aorta, orbits, salivary gland, thyroid, mediastinum and skin. The characteristics are elevated serum IgG4 levels, dense lymphoplasmacytic infiltrate rich in IgG4 positive plasma cells and storiform fibrosis by histologic findings. In case of renal involvement, that is usually accompanied by symptoms such as fever, fatigue or flank pain, abnormal urinalysis, or increased serum creatinine. However, IgG4-related renal disease without renal manifestation, involving the nervous system is not common. Herein, we report a case of IgG4-related renal disease without renal symptoms presenting mass-like lesions along the bilateral trigeminal nerve. A 71-year-old male was admitted to the hospital because of bilateral facial numbness over 6 months. He had a history of autoimmune pancreatitis treated with steroid in 2010. Magnetic resonance imaging (MRI) revealed two lobulating enhancing masses along the bilateral trigeminal nerve, and the lesions were considered by trigeminal schwannoma. He was performed by enhanced computer tomographic (CT) scan. CT finding showed that large irregular enhancing soft tissue masses at renal pelvis involving bilateral proximal ureter, centrilobular nodules on both lower lung fields and enlarged mediastinal lymph nodes. Laboratory findings showed normal serum creatinine and urinalysis, but increased serum IgG, IgG4 levels and hypocomplementemia. Renal biopsy revealed diffuse interstitial lymphoplasma cell infiltration with fibrosis and IgG4 positive plasma cells counted more than 10 per 1 high power field. The patient was initially treated with glucocorticoid. The treatment improved the symptoms and masses on abdomen CT and brain MRI.