Distinguishing immunoglobulin G4 (IgG4)-related disease ocular involvement (IgG4-ROD) from other non-IgG4 pathologies in the orbit and eyeball is often complex. The objective was to compare clinical, analytical, imaging, and anatomopathological features of IgG4-ROD with other inflammatory and/or autoimmune ocular diseases (non-IgG4-ROD).