UNLABELLED: The congenital absence of multiple teeth may share the same genetic background of the development of some types of cancer. OBJECTIVE: This systematic review aimed to investigate the possible association between dental agenesis and cancer, and the perspective of agenesis as an early predictor for cancer risk. METHODOLOGY: The electronic databases PubMed, Scopus, Web of Science, Cochrane Library, LILACS, and OpenGrey were searched and the risk of bias was evaluated using the Newcastle-Ottawa tool. The GRADE tool was used to evaluate the certainty of the evidence. RESULTS: Six studies met the eligibility criteria. A positive co-occurrence between ovarian cancer and hypodontia was found in two articles. Three studies evaluated the association between dental agenesis and colorectal cancer and only one showed common genes for these conditions. One paper found individuals with hypodontia had a higher risk of family history of cancer. Five studies had a fair quality and one a good quality. The certainty of evidence was classified as very low. CONCLUSION: Notwithstanding the limited scientific evidence, there may be a possible association between dental agenesis and cancer due to genes involved in both conditions. Agenesis of multiple teeth could be an early indicator of cancer risk. Nevertheless, studies with a better level of evidence are needed to confirm this possible association.