Tooth agenesis–colorectal cancer syndrome, also known as oligodontia–colorectal cancer syndrome (ODCRCS) is a rare congenital disorder which is characterized by absence of at least six teeth and an increased risk of malignancies like colorectal or breast cancers (Lammi et al. 2004). It was first described in 2004 by Laura Lammi and colleagues in a Finnish family with oligodontia and colorectal neoplasia or precancerous lesions (Lammi et al. 2004). Recently it was found that it can be presented with cleft palate (Roht et al. 2023). The prevalence of ODCRCS is estimated to be less than one per million people (Roht et al. 2023). The patients have a wide range of age from 4 to 95 years old (Roht et al. 2023), while the age of onset has not still determined clearly.