Abstract： IgG4-related disease (IgG4-RD) is a systemic inflammatory disease which is characterized by chronic inflammation and fibrosis in the involved tissues/organs, predominantly infiltrated with lymphocytes and plasma cells. The respiratory system is usually involved in IgG4-RD, however, the respiratory clinical manifestations are varied from case to case, and they might mimic other respiratory diseases. We reported a patient who had been diagnosed with severe asthma for around 7 years. He was also suffered from allergic rhinitis. He had peripheral eosinophilia and elevated serum total IgE and IgG4. Finally, he was diagnosed with IgG4-RD after submaxillary gland resection and cervical lymph node biopsy. His condition was improved significantly after oral corticosteroids and immunosuppressants.