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Abstract Idiopathic normal pressure hydrocephalus (iNPH) is a subtype of hydrocephalus that occurs more often in the elderly population. It is usually characterized by gait disturbance, dementia and urinary incontinence. Epidemiological studies indicate that 15.7–17.8% of iNPH patients present with type-2 diabetes mellitus (DM). A review of the primary literature shows that these occurrence rates are higher than age- and cohort-matched non-iNPH controls. This suggests that this already vulnerable patient group has an increased risk for presenting with DM compared to their non-iNPH counterparts. Postoperative outcome when treating iNPH patients is inversely related to the number of patient comorbidities and a lower comorbidity status is correlated with better outcomes. This review highlights the need for further research into the relationship between iNPH and DM and speculates on a possible mechanism for an association between the development of ventriculomegaly and the development of DM and iNPH.