Gender dysphoria (GD) in children and adolescents is a condition that is characterized by an incongruence between the assigned and experienced gender. Despite the diversity in clinical presentation, literature demonstrates that GD might lead to poor mental health and high rates of co-occurring psychopathology. Due to the overlap of physical aspects as well as psychological needs in these children, a multidisciplinary approach is highly desirable. The aim of this narrative review is to give an overview of recent literature on several topics relevant in this domain. Guidelines on psychological counseling and hormonal treatment are given and challenging topics subject to controversy are explained. Furthermore, attention is drawn to the risks and protective factors in psychological functioning, including the growing evidence of a frequent co-occurrence with Autism Spectrum Disorder. Finally the psycho-sexual development in these children, the impact on fertility and fertility preservation are discussed.