AIM To review the efficacy of intraorbital corticosteroid injection for the treatment of IgG4-related ophthalmic disease (IgG4-ROD). METHODS This study was a retrospective twin-centre histopathology review and observational case series. A chart review was undertaken for 10 cases of biopsy-confirmed IgG4-ROD treated with intraorbital corticosteroid injection. The main outcome measures were the magnitude, onset and duration of the clinical response. RESULTS All cases received intraorbital injection(s) of 20 or 40 mg doses of triamcinolone acetonide suspension (Kenacort-A 40, Bristol-Myers Squibb, Australia). Intraorbital corticosteroid injection was associated with a swift positive response in all cases. 50% of cases achieved complete and sustained clinical remission during mean follow-up of 41 months. This was achieved with a single injection in three cases and with repeat injections in two cases. Peak clinical improvement was reached in ≤7 days following 61% of injections. 60% of cases suffered relapse and 56% of relapses occurred during weeks 3 or 4 following injection. Sclerosing histology was associated with a clinical response that was gradual in onset (p=0.01) and that tended to be incomplete (p=0.4). There were no complications of injection other than brief discomfort. CONCLUSIONS Intraorbital injection of corticosteroid may be a useful treatment option for IgG4-ROD. It may have a role as a first-line therapy in selected cases of IgG4-ROD centred in the anterior orbit. However, most cases require repeat injections due to incomplete response and relapse.