Background: Ambiguous genitalia represent a sequel of sexual development disorders where the external genitalia don’t have a typical resemblance to that of male or female. Methods: This was a female case of ambiguous genitalia, her age was twenty-eight years, ultrasound examination revealed atrophied uterus and bilateral undescended testes. She complained of gradual size increase of her left testicle in the last 6 months. Due to sudden trauma, she developed severe pain and swelling in her left groin. On examination: patient looked unwell, in pain, afebrile, locally there was swelling about 10 cm in size with shiny and red looking, it was very tender, otherwise it was unremarkable. So, this was considered as a case of left testicular trauma that need urgent left groin exploration. The patient was counselled and consented about possible orchidectomy. Intra-operative note: under spinal anesthesia patient was put on supine position, skin painted by yamidin, left inguinal incision was made and hematoma was evacuated, the left testicle was found to be hugely enlarged and ruptured. The spermatic cord was clamped and orchidectomy was done. No intra operative complications. Result: Histological examination of the testicular specimen confirmed that it was a germ cell tumor [Seminoma]. Patient was recommended to for: (1) Chemotherapy at the Oncology Department, (2) Laparoscopic search for her right undescended testis to confirm whether it is a testis or an ovary, (3) Buccal biopsy for sex determination and (4) Surgery, for the right side, if needed. Conclusion and recommendation: Neglected cases of undescended testis may have a serious outcome. Screening for ambiguous genitalia, and early exploration and management was highly recommended.