Gender dysphoria in individuals with schizophrenia may result from the delusionally changed gender identity or appear regardless of psychotic process. Distinguishing between these situations is not only a diagnostic challenge, but also affects the therapeutic decisionmaking. The review of the literature shows that different delusional beliefs regarding belonging to another gender, anatomy or changes within the genitals affect about one-fourth of patients with schizophrenia. Contemporary classifications of disorders are moving towards the elimination of psychotic disorders as a disqualifying criterion in diagnosing gender dysphoria. It is also established that schizophrenia may change the picture of gender dysphoria, e.g., by giving meaning and delusional interpretations of the fact of the incompatibility of phenotypic sex with the sense of gender. At the same time, before making a therapeutic decision (especially aimed at gender reassignment), it is necessary to exclude the psychotic background of the desire for gender reassignment. In case of co-occurrence of both disorders, it is crucial to evaluate the chronology and dynamics of the individual symptoms, their constancy (prolonged observation), patient’s criticism and response to antipsychotic treatment.