Immunoglobulin G4-related disease (IgG4-RD) is a rare and complicated clinical disease entity newly identified in recent years. It is a chronic inflammatory disease mediated by the immune system that can affect various organs throughout the body, such as the pancreas, salivary gland (submandibular gland, parotid gland, and sublingual gland), lacrimal gland, and thyroid gland. In rare cases, it involves sinuses and orbits. We describe a 44-year-old patient who was hospitalized with distension and pain in her right eye. The lesions included the nasal sinus and orbit. IgG4-RD was diagnosed after surgery and the patient was treated without complications. This case report helps to provide clinicians with additional information to assist in the diagnosis of the disease.