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Primary care providers who encounter children are often the first line of contact for individuals with gender dysphoria, which occurs when sex assigned at birth is incongruent with one's true, expressed sexual identity. Because those with untreated gender dysphoria are at risk of a variety of negative outcomes, including mood symptomatology, suicidality, substance use disorders, and other psychosocial risk factors, it is critical that health care providers are adept in the provision of holistic, patient-centered care. The purpose of this report is to provide an updated review of the current evidence from the literature pertaining to the identification, treatment, and coordination of care among children with gender dysphoria within the primary care setting or medical home.Using PubMed and CINAHL, a literature review spanning from 2012 to the present was conducted using the following key words: gender dysphoria, transgender health, LGBT health, and hormone therapy. Reference lists of identified articles were also explored for relevance.Treatment may include a social transition, hormone antagonist therapy, or the administration of cross-sex hormone therapy, with a medical home needed to facilitate coordination of care. Best practice guidelines vary across pediatric and developmental groups and include both reversible and nonreversible modalities. Screening for negative psychosocial sequelae must be completed to include mood symptomatology, suicidality, substance use disorders, and risky sexual behavior, so that appropriate screening, identification, and treatment interventions can be implemented.The primary care medical home must act as a foundation for the identification of gender dysphoria and/or associated comorbidities and must treat, when able, or refer, when indicated. In addition, because of structural barriers and stigmatization, public policy often fails the transgender community and can exacerbate the aforementioned psychosocial comorbidities faced by the transgender youth community. Health care providers, particularly nurse practitioners, are in a unique position to expand on the face-to-face care provided to the community and engage in advocacy efforts to dismantle structural barriers impeding transgender individuals and communities while also providing primary health care, anticipatory guidance, and care coordination.