Over the past decade, more and more children and adolescents are identifying as transgender and gender diverse (TGD). Often, they and their parents first turn to their primary care pediatrician for guidance and support. Therefore, in 2018, the American Academy of Pediatrics (AAP) released a policy statement focusing on the health care of TGD youth. The AAP acknowledges that many pediatricians have a lack of training in this area and therefore need to increase their knowledge base and expertise in order to provide culturally competent care. While most sexual and gender minority individuals are healthy and well adjusted, some TGD youth are at an increased risk of mental health concerns, including anxiety, depression, substance abuse, eating disorders and suicidality. This is theorized to be due to the experienced or internalized marginalization, stigmatization, victimization, harassment or rejection and not inherent in having gender dysphoria or being transgender. The pediatrician therefore needs to be knowledgeable of, and skilled to screen for, the health disparities that may exist, as well as to be able to support the individual who may disclose their gender identity status during treatment. Parents and guardians may also turn to the pediatrician for guidance when faced with their child who presents with gender non-conforming behaviors or gender dysphoria. Therefore the pediatrician needs to be able to guide the parents as well, as their acceptance and support of their child's journey to gender identity is probably the most important protective factor against health disparities encountered. To deliver optimum care for TGD children and youth, the pediatrician needs to establish an office setting that is inclusive, gender-neutral and TGD friendly. By becoming familiar with the diversity of gender expression and identities, use of gender-appropriate terminology, health disparities often encountered and the importance of providing a safe and welcoming environment, issues that are all covered in this article, the primary care pediatrician will be in position to provide comprehensive health care to this often marginalized population.