Exfoliative erythroderma is a severe dermatologic condition with variable underlying causes. The association of erythroderma with an occult visceral malignancy is a relatively uncommon finding, which, however, can be accompanied by devastating consequences. This report is the first updated overview regarding the latest data on paraneoplastic erythroderma and was conducted according to the PRISMA Guidelines for systematic reviews. Articles derived from the databases PubMed, EMBASE, and SCOPUS were analyzed for this study. Patients with paraneoplastic erythroderma share certain clinical characteristics that physicians should be aware of, so as to avoid potential sequeale of an erroneous management as a result of misdiagnosis. The reporting of such cases is crucial in order to raise awareness of this condition and to facilitate an earlier diagnosis.