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Background/Objectives Mitochondrial cytopathies are complex heterogenous multi-system diseases that commonly cause diabetes through a variety of mechanisms. Despite this, little is known about the ideal management of diabetes and its complications in these patients.Methods We conducted a retrospective review of patients from the mitochondrial clinic at Neuroscience Research Australia (NeuRA), to elucidate what current treatments are being used in this cohort and examine the rates of serious side effects. Data collected included genetic diagnosis, medication history, and medical co-morbidities including chronic kidney disease and heart failure. We also performed a literature review comparing our experience to other mitochondrial cohorts.Results We reviewed 142 patient records. 4/142 (2.8%) had type 1 diabetes and 19/142 (13.4%) had type 2 diabetes. Of the patients with diabetes (n=23), 14 (60.9%) are on insulin, 9 (39.1%) are on metformin, 7 (30.4%) are on SGLT2 inhibitors,5 (14.7%) are on sulfonylureas, 4 (17.4%) are on GLP-1 inhibitors and 4 (17.4%) are on DPP-4 inhibitors. No serious side effects were reported.Conclusion/Discussion Our review characterises the real-world experience of diabetic pharmacotherapy in mitochondrial disease. Interestingly, metformin was the second most frequently used agent without any reports of severe lactic acidosis. Prospective data examining glycaemic control, cardio-renal benefits and tolerability of different classes of diabetic pharmacotherapy would help inform best clinical practice in mitochondrial patients.