The phenomenon of atypical gender identity in adolescents and adults, in diagnostic classifications called gender identity disorder – transsexualism (ICD-10) or gender dysphoria (DSM-V), arouses interest among research communities and has been reported in the Polish and foreign literature. At the same time, much less attention is given to the very same phenomenon occurring in childhood, leaving a gap in the area of knowledge and competence potentially significant to a wider range of specialists including psychiatrists, psychotherapists, paediatricians, and social workers. The aim of this paper is to provide a synthesis and present the current state of knowledge about the situation of children with atypical gender identity and their families. Available data pertaining to aetiology, psychopathology, and developmental paths of individuals experiencing gender dysphoria of childhood are analysed. Moreover, the aim is to draw attention to controversies surrounding diagnostic and therapeutic processes and significant, i.a., in the context of the observation that the vast majority of gender-dysphoric (GD) children appear to no longer experience the symptoms during adolescence and adulthood. Therefore, the paper presents some important areas of assistance and stresses the need for great caution and individual approach when working with GD children and their families.