OBJECTIVES: Cardiovascular diseases (CVD) constitute major comorbidities in type 2 diabetes (T2DM), contributing to substantial additional treatment costs. An updated overview of its cost impact has not been presented. Our objective was to systematically review published articles describing the costs associated with treating CVD in people with T2DM. METHODS: Two reviewers independently searched Medline, Embase, and abstracts from scientific meetings for original research published between (2007-2017), with no restrictions placed on publication language. Studies reporting direct and/or indirect costs of T2DM at either a macro level (e.g., burden of illness for a country) or micro level (e.g., cost incurred by one patient) were included. Extracted costs were inflated to 2016 values and converted to USD.RESULTS: Out of 81 articles identified, 24 were accepted for analysis, of which 14 were full articles and 10 abstracts. Studies from 13 countries were included: Brazil, Croatia, Estonia, India, Italy, Japan, Mexico, Scotland, South Korea, Spain, Sweden, United States, and the United Kingdom. CVD comorbidities in patients with T2DM incurred a significant burden both at a population and patient level. Two studies reported results from a population level. CVD costs contributed from 32%-49% of the total direct costs of treating T2DM (weighted average=42%). From a patient level, compared to patients with T2DM without CVD the median annual cost per patient for cardiovascular disease, coronary artery disease, heart failure and stroke were 112%, 107%, 59%, 322% higher, respectively (average=91%). Two studies reported indirect costs of 37% (Brazil) and 60% (UK) of total costs. Discount rates were not reported in any of the studies. A description of the model/type of economic evaluation undertaken was described in 75% of the studies. CONCLUSIONS: Globally, CVD exerts a substantial additional costs to the treatment of diabetes for both healthcare systems (+42% to total cost) and per patient (+91% annually).