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The ketogenic diet (KD) is a low-carbohydrate, high-fat, adequate-protein diet proven to be effective for the reversal of obesity, metabolic syndrome and type 2 diabetes, and holding therapeutic potential for the prevention and treatment of other chronic diseases. Genetic and dynamic markers of KD response may help to identify individuals most likely to benefit from KD and point to individuals at higher risk for adverse health outcomes. Here, we provide a clinician-friendly review of state-of-the-art research on biomarkers of KD response for a variety of outcomes including weight loss, body composition and cognitive performance drawing data from both intervention trials and case reports of rare inborn errors of metabolism. We also present a selection of the most promising candidate genes to evaluate in future studies and discuss key aspects of study design and variant interpretation that may help accelerate the implementation of these biomarkers in clinical practice., Competing Interests: Competing interests: JV is a founder and equity holder in Virta Health, scientific advisors for Atkins Nutritionals and UCAN, and receives royalties from books on ketogenic diets. AP is coowner of the company Poff Medical Consulting and Communications which provides scientific consulting services. AP is also coowner of the company Metabolic Health Initiative, LLC which hosts the annual scientific conference Metabolic Health Summit. AP is an inventor on patents related to ketone technology. DPD is an inventor on International Patent # PCT/US2014/031237 University of South Florida: Compositions and Methods for Producing Elevated and Sustained Ketosis. DPD is a coowner of the company Ketone Technologies, a company specialised in scientific consulting., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)