Tehran University of Medical Sciences (TUMS) Academic Journal of Surgery 2423-3218 4 1 2017 07 25 24 26 EN Shahram Shabaninia Assistant Professor, Department of Urology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran Seyed Reza Yahyazadeh Assistant Professor, Department of Urology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran 2017 04 19 2017 07 25 Background: Müllerian duct cyst is an uncommon congenital anomaly. It is usually small and asymptomatic. A rare case of this entity with symptoms of ejaculatory duct obstruction such as hematospermia and secondary infertility is presented here. Case Report: A 39-year-old male presented with complaints of pain in the lower abdomen, dysuria, frequency, and hematospermia for the last 2 years. He also suffered from secondary infertility with a 9-year-old son. Imaging studies showed a large cystic midline lesion right behind the bladder and also bilateral dilated ejaculatory ducts. The patient underwent a transurethral drainage. The prostatic urethra was resected using a 26 Fr resection loop proximal to the verumontanum and a capacious cystic structure opened, and finally two dilated ejaculatory duct openings were seen. On follow-up, a significant improvement of symptoms and semen quality was achieved after surgery. Conclusions: A high index of suspicion in using advanced imaging modalities is necessary for the diagnosis of this anomaly. Surgical excision of a Müllerian duct cyst may be performed depending on the size and location of the cyst and the presence of clinical symptoms. Transurethral de-roofing of the cyst is effective and safe for the treatment of small Müllerian duct cyst accompanied with ejaculatory duct obstruction. https://ajs.tums.ac.ir/index.php/ajs/article/view/88 https://ajs.tums.ac.ir/index.php/ajs/article/download/88/59