Understanding the root-canal system is vital and benefits canal cleanliness, shape, and block when performing root canal treatment. However, tooth genesis and development are regulated by genes and affected by the environment. Taurodontism, a rare tooth anomaly, is featured with enlarged pulp chamber, apical displacement of the pulp floor, root bifurcation, and variations in root canal. Dealing with this disease through root canal treatment is challenging and prognosis is uncertain. Thus, taurodontism diagnosis and treatment are important. This article focuses on taurodontism pathogenesis, diagnosis, classification, differential diagnosis, treatment, and prognosis and summarizes some views for dentists.