The objective of this study is to analyze the relationship between chronic rhino-sinusitis (CRS) and systemic symptoms in patients with IgG4-related disease (IgG4-RD). The patients with IgG4-RD, confirmed by restrict association with clinical and histopathological manifestations between March 2013 and July 2016, were enrolled and followed-up for 1 year at the Tongren Hospital, Capital Medical University. The patients were divided into two groups: the case group included IgG4-RD patients with CRS confirmed by clinical and imaging, while the control group included IgG4-RD patients without CRS confirmed by clinical and imaging. Age, gender, clinical manifestations, the percentage of eosinophils in peripheral blood, sedimentation (ESR), C-reaction protein, serum IgE and IgG4 levels, histopathology, and treatment drugs at the baseline and 1 year of follow-up were compared between the two groups. A total of 46 cases met the diagnostic criteria for IgG4-RD. A total of 30 patients (65.2%) had IgG4-RD complicated with CRS, and were aged 49.7 +/- 13.4 years, with male:female ratio = 2:1. The disease duration in the case group was longer than that in the control group (3.0 versus 0.8, p = 0.009). The ratio of ocular involvement was higher (86.7 versus 60%, p < 0.001), and allergic manifestations including drug allergy, asthma, and allergic skin were more common (56.5 versus 20%, p = 0.004), with a higher percentage of eosinophils in peripheral blood (8.5 versus 3.3%, p = 0.018) and more sensitive to glucocorticoids (6.0 versus 3.5, p = 0.004) than those in the control group. CRS in patients with IgG4-RD was closely associated with IgG4-related ocular lesions, which was more prone to allergic manifestations accompanied by raised percentage of eosinophils in peripheral blood. The treatment of patients with IgG4-RD complicated with CRS was more effective than those with IgG4-RD without CRS.