The authors set out to analyse the effect of acetazolamide therapy on inflammatory cystoid macular oedema (CMO) as defined by change in central macular subfield thickness (CST), as well as its effect on visual acuity. A retrospective chart review was performed of patients treated with acetazolamide or methazolamide off-label for macular oedema related to uveitis and Irvine-Gass syndrome. A total of 16 patients (19 eyes) met the inclusion criteria. At the time of clinical activity index (CAI) initiation, 7/16 (44%) patients were taking systemic prednisone and / or other immunomodulators, and 14/19 (74%) eyes were being treated with topical steroid drops. 13/16 (81%) patients reported adverse effects from CAI therapy. These included paresthesias (n=10), dysgeusia (n=6), fatigue (n=5), and diarrhoea (n=3). A total of 13/19 (68%) eyes demonstrated a clinically significant decrease in CST or total resolution of their CME after the addition of acetazolamide. Mean visual acuity improved at first follow-up. In the current case series, no clear predictors of response to therapy as judged by improved CST were identified using the OCT analysis. Addition of acetazolamide therapy to patients with recalcitrant inflammatory CME can provide a significant anatomical and functional benefit.