Magnetic Resonance Imaging Findings of Ovotesticular Disorder of Sex Development with Bilateral Gonadoblastoma

Ovotesticular disorder of sex development (DSD) is a very rare chromosomal anomaly characterized by the presence of both ovarian and testicular tissues.
We reported a case of delayed diagnosis in a 18-year-old man presenting with nonpalpable testes. Pre-operative magnetic resonance imaging (MRI) demonstrated associated anomalies (agenesis of seminal vesicle, configuration of urethra) and Mullerian duct remnants. The presence and location of both ovary and testis, and restricted diffusions of gonads were detected on MRI. On histopathology, malignancies were found in both gonads.
Pre-operative MRI findings were helpful for surgical approach. We emphasized the importance of pre-operative MRI on treatment planning.