Background: Common potentially oral malignant disorders consist of leukoplakia, erythroplakia, submucous fibrosis, and lichen planus. Among these lesions, erythroplakia has been identified as the one with the highest malignant potential. The diagnosis and treatment of oral erythroplakia (OE) is always an important issue clinically. Methods: The purpose of this review is to provide the detailed information of literature review and update of etiology, morphology, subsites, size, histopathology, molecular biology, treatment outcomes, and modern new optic technique of OE. Results: Early detection, correct diagnosis, and adequate treatment are the key steps to a successful outcome and good prognosis. Clinical course of such lesions is variable and the postoperative recurrence is unpredictable. Conclusions: OE is characteristic of high percentage of carcinomatous lesion harbored inside and high potential for malignant transformation, but it can be successfully managed by surgery. Patients with OE are suggested to attend a regular follow-up at short intervals of time regardless of whether the treatment is given or not.