Trichodentoosseous (TDO) syndrome is a rare autosomal dominant condition characterized by various dental and non-dental findings such as taurodontism, amelogenesis imperfecta, osseous dysplasia, mandibular prognathism, curly hair, dysplastic nails, which may be symptomatic or asymptomatic. TDO syndrome is divided into three subtypes that helps to categorize different features seen in patients. There are very few cases reported in the literature of TDO syndrome. We present a case of a young adult male showing interesting Type I and II clinical and radiographic findings of the TDO syndrome. Amelogenesis imperfecta hypomaturation-hypoplastic type and TDO syndrome overlaps in their dental findings such as taurodontism and enamel hypoplasia and makes the diagnosis of TDO crucial. TDO syndrome was noted as an incidental finding on cone beam CT. This case report highlights the pathognomonic radiographic findings, treatment plan, and the clues to diagnosis this rare disorder. Management of TDO requires a proper diagnosis, multidisciplinary approach with comprehensive treatment plan including periodic follow up. Knowledge of this condition along with thorough interpretation of the entire cone beam CT volume are critical to understand this syndrome better due to its rarity.