Purpose: To further characterize the clinical presentation, diagnosis, and current treatment strategies of IgG4-related ophthalmic disease. Methods: Review and analysis of seven cases orbital inflammatory syndrome found to be biopsy positive for IgG4. Results: We present seven cases of IgG4-related ophthalmic disease, a previously unknown entity that has only recently been identified as a treatable systemic disorder. Management included surgical excision, systemic steroids, steroid-sparing immunosuppression, and radiotherapy. Conclusions: These cases illustrate the importance of including IgG4-related ophthalmic disease in the differential diagnosis of periorbital or orbital masses. Prompt recognition of this entity by clinicians can allow for effective treatment with corticosteroids. Treatment with steroid-sparing immune suppressants and radiotherapy has shown promise in chronic or refractory disease, nonetheless tumor excision may ultimately be necessary.