AbstractIgG4-related disease (IgG4-RD) is a condition that was first described recently, and is capable of affecting any organ of the body. Diagnosis is based on the correlation of clinical findings with histopathological findings and elevated serum IgG4. Treatment involves corticosteroids and rituximab for the most severe cases. We report the case of a symptomatic patient diagnosed of IgG4-RD whose diagnosed was guided by elevated serum IgG4 levels.