BACKGROUND: Most cases of tooth agenesis are presented in the form of hypodontia with different patterns of missing teeth. In addition, there is a frequent clinical association between tooth agenesis and other anomalies. OBJECTIVE: To systematically analyze the literature on the prevalence of agenesis of permanent teeth associated with other dental anomalies in nonsyndromic patients. MATERIAL AND METHODS: An electronic search was performed in seven primary databases (Embase, LILACS, BBO, MedLine via PubMed, SciELO, Scopus, and Web of Science) and two additional ones (OATD, and ProQuest) to partially capture the "gray literature." The PECO strategy was used to identify observational studies evaluating the prevalence of agenesis of permanent teeth associated with other dental anomalies in nonsyndromic patients, without restrictions on publication language or year. Two reviewers extracted the data and assessed the individual risk of bias of the eligible studies using the JBI Critical Appraisal Tools for use in JBI Systematic Reviews - Checklist for Cross-sectional, Case-Control, and Cohort Studies. RESULTS: The electronic search revealed 8659 records, seven of which met the eligibility criteria, and were included in the qualitative synthesis. The studies were published from 1961 to 2024. Patients with agenesis were more likely to present taurodontism, microdontia, enamel hypoplasia and distoangulation of the second premolar. Ectopic eruption, infraocclusion of the first deciduous molar and impacted tooth showed mixed results among the eligible studies. CONCLUSION: Based on limited evidence, tooth agenesis was positively associated with the prevalence of other concomitant tooth agenesis.