Purpose <p>Psychiatric presentations and psychopathology in people with co-occurring gender diversity (GD) and autism spectrum disorder (ASD) are not well understood. The aim of this study is to characterize mental health presentations and history (psychiatric diagnoses, psychotropic medication use, history of suicidal ideation and suicide attempts, and psychiatric hospitalization rates) in patients with co-occurring ASD and GD among those seeking psychiatric care at a tertiary care outpatient neurodevelopmental disorders center.</p> Methods <p>This retrospective chart review included 125 patients who were divided into three study groups: index patients with co-occurring GD and ASD (<i>n</i> = 25), and age-matched comparison males (<i>n</i> = 50) and females (<i>n</i> = 50) with ASD and without indication of GD in their medical records. All subjects were required to have one initial psychiatric note documented in their electronic medical record (EMR), and their records were reviewed for psychiatric diagnoses, psychotropic medication use, history of suicidal ideation and suicide attempts, and psychiatric hospitalizations.</p> Results <p>All three groups experienced high rates of psychopathology across all characteristics of presentation and history assessed, aside from psychiatric hospitalization which was infrequent across groups. 119/125 (95%) subjects were assigned two or more psychiatric diagnoses in addition to the ASD diagnosis required for study eligibility.</p> Conclusion <p>Results warrant further investigation of the prevalence of psychiatric conditions in GD individuals with ASD.</p>