Background and Objective: Within the past decade, more transgender and gender diverse youth that experience gender dysphoria seek transition -related medical care, thus there is a growing and continued need to improve knowledge and expertise in this topic. This narrative review aims to provide a comprehensive discussion of current definitions and diagnostic criteria related to gender dysphoria, understanding that determinants of an individual's gender identity is likely influenced by multiple constructs, and providing the readers with an approach to clinical recognition and evaluation of a child or adolescent with gender dysphoria. Methods: We conducted a PubMed search including literature in the English language using key words: gender dysphoria, transgender, gender -diverse youth, gender identity. We considered publications between 1995-2022. Specifically, publications from 2012-2022 were considered to support recommended approach to evaluation for gender dysphoria. Key Content and Findings: There is increasing evidence on the contributions of genetics, hormone influences and neurobiology on gender identity. Furthermore, data on the benefits of access to gender affirming therapy continue to emerge. Transgender youth are at increased risk of psychiatric comorbidities such as depression, anxiety, and life -threatening behaviors. As such, studies underscore the importance of early recognition, mental health support and medical intervention through hormone treatments for pubertal suppression and/or gender -affirming hormone therapy in decreasing mental health burdens and improving quality of life among the transgender youth. Conclusions: While there have been significant strides in our understanding of gender -related issues in youth, there remains a significant gap in knowledge related to medical care, and how the medical community can further support these individuals. Further studies on the biological basis of gender diversity, continuing to explore genetic, endocrine, and neuroanatomic influences, may aid in redefining gender constructs and decrease social stigmas associated with it. Guidelines and approach to care for transgender youth are now more widely available, primarily aimed at initial identification and intervention, but with strong recommendations of continuing multidisciplinary care that will provide support and expertise during the physical and psychosocial interventions, while ensuring continued communication and discussion of treatment recommendations and ongoing management.