Background: Gender dysphoria (GD) appears disproportionately common in people with autism spectrum disorders (ASDs). Concurrent intellectual disability (ID) may limit assessment of these individuals and their access to gender-affirming healthcare. This study aims to summarise knowledge on the presentation of GD in those with co-occurring ASD and ID. Methods: A systematic search was conducted of five online databases from 1984 to September 2021, for articles pertaining to GD in people with ASD and ID. Additional studies were obtained from personal research. Results were screened against defined eligibility criteria, with data extracted on participant characteristics and clinical presentation. Results: From 149 total articles, 10 were included (case studies (n = 4), cross-sectional studies (n = 2), systematic reviews (n = 4)). Conclusions: GD does affect this population and can cause significant distress; two cases reported subsequent self-harm or suicidality. Further conclusions were limited by the paucity of relevant literature and further research is required.