Introduction: True hermaphroditism, also known as mixed gonadism, is a rare genetic anomaly manifested by the presence of both male and female gonads, giving the individual a mixed phenotype and thus sexual ambiguity. The objective was to demonstrate the value of imaging in this pathology through an observation in our department. Observation: This was an 18-year-old subject with no known history, claiming to be male. He was admitted to the Marie Curie medical clinic for sexual ambiguity. Clinically, there was gynecomastia, a female-type pelvis with a rudimentary vulva, and female-type suprapubic hair. Primary amenorrhea, recurrent pelvic pain, ejaculation, and morning erections were noted. A micropenis without a urethral meatus in place of the clitoris was present. There was no vulvar vestibule or labia minora. Biologically, serum testosterone was normal (3.6 ng/ml), serum FSH was normal (11UI/L), and LH was abnormal (11.4 IU/l). Pelvic ultrasound and magnetic resonance imaging revealed a right ovary, a uterus, a left testicle, and structures similar to the corpus spongiosum and cavernosum of the penis. Molecular testing revealed the XX genotype. Conclusion: Cross-sectional imaging remains essential in the management of true hermaphroditism.
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