Ketogenic diets are a widely known, yet controversial treatment for cancer patients. In this review, we summarize the clinical evidence for anti-tumor effects, as well as the effects on anthropometry, quality of life, adverse events and adherence in cancer patients. In April 2019, a systematic search was conducted searching five electronic databases (EMBASE, Cochrane, PsychInfo, CINAHL and Medline) to find studies analyzing the use, effectiveness and potential harm of a ketogenic diet in cancer patients of any age as sole or complementary therapy. From all 19.211 search results, 46 publications concerning 39 studies with 770 patients were included in this systematic review. The therapy concepts included all forms of diets with reduced carbohydrate intake, that aimed to achieve ketosis for patients with different types of cancer. Most studies had a low quality, high risk of bias and were highly heterogeneous. There was no conclusive evidence for anti-tumor effects or improved OS. The majority of patients had significant weight loss and mild to moderate side effects. Adherence to the diet was rather low in most studies. Due to the very heterogeneous results and methodological limitations of the included studies, clinical evidence for the effectiveness of ketogenic diets in cancer patients is still lacking.