Although the attention for substance use (SU) and SU disorders (SUD) among individuals with mild to borderline intellectual disability (MBID) has been growing exponentially, this form of dual diagnosis has largely been ignored by addiction medicine. In this article, we systematically review the research between January 2000 and June 2018 on the prevalence, assessment, and treatment of SU(D) among children, adolescents, and adults with MBID. A total of 138 articles were included. It is concluded that individuals with MBID are likely to be at a higher risk for developing SUD compared to those without MBID. Future research should focus on the detection of MBID among patients being treated in addiction medicine, the development and implementation of systematic assessment methods of SU(D) among individuals with MBID, and the development and evaluation of prevention and treatment interventions. System integration, interdisciplinary collaboration, and the development of tailored treatment for individuals with MBID are advised to improve treatment access and outcome for those who have developed SUD.