The frequency of testicular cancer in men with intellectual disabilities is not precisely known, with the exception of some genetic conditions such as Down syndrome, where it has increased. Objective: To review systematically the literature through Pubmed with a particular focus on epidemiological studies of testicular cancer in persons with intellectual disability. Method: Literature review. Results: Testicular cancer was more frequent in subjects with intellectual disability than in the general population. Yet the occurrence depended on the specific genetic condition. The study of the association with not genetically determined intellectual disabilities remains to be done. Conclusion: Clinicians in charge of men with intellectual disability should be aware of the association with testicular cancer.