Objectives: Dental agenesis — a congenital lack of teeth — is one of the most frequently diagnosed developmental defects of dentition. Genetics is a crucial factor in the etiology of this disorder. Missing teeth can be caused by mutation in genes including MSX1, PAX9, AXIN2, and EDARADD. As is also true for ovarian cancer, over 20% of cases are associated with hereditary factors. Mutations in the BRCA1 and BRCA2 genes are said to be the most frequent of these. The aim of this study was to provide a systematic review of the literature on the coexistence of ovarian cancer and tooth agenesis. Material and methods: Publications were searched for in the online databases PubMed, SCOPUS, and Wiley Online Library. Current and archival issues of the Journal of Stomatology and Dental and Medical Problems were also searched. The key words used to find relevant publications were: ovarian cancer, hypodontia, and tooth agenesis, in various combinations. Results: Three publications were qualified to this review. Two of these compared the incidence of hypodontia in women with ovarian cancer and in healthy women, and the other was aimed at locating the gene responsible for the coexistence of ovarian cancer and tooth agenesis. As shown by these studies, women with ovarian cancer are (depending on the study) 3.3 or 8.1 times more likely to have hypodontia than healthy women. However, no specific gene was found that might be responsible for the coexistence of ovarian cancer and tooth agenesis.