Background: We present a case an adolescent female patient with Gender Dysphoria (GD) who was diagnosed with a recurrent ovarian neoplasm - Juvenile Granulosa Cell Tumor (JGCT).Case presentation: A 17-year-old female patient presented multiple endocrine pathologies and a recurrent JGCT. During the surgery qualification process, the patient admitted to identifying herself as a male. The patient described being uncomfortable with her body and with the expected roles of her assigned gender. Due to that, the patient requested a total hysterectomy with a bilateral salpingo-oophorectomy. As a minor, she required parent permission, which was not granted. The patient underwent several specialist consultations, after which she agreed for unilateral removal of tumor-changed pathologies and additional hormonal, psychological, and psychiatric diagnostics.Conclusion: To the best of our knowledge, this is the first detailed report of co-occurrence of GD spectrum disorders and JGCT in an adolescent female. This case contains many therapeutic and ethical problems regarding both physical and mental health. It should be noted that adolescents with the GD spectrum rarely develop persistent transsexuality. Modulations from developmental psychology, psychotherapy, family dynamics, hormonal treatment, and the removal of JGCT in the presented case may have potential therapeutic implications for GD.