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A 2 years old boy was admitted in our institution for abdominal mass. He presented a growing abdominal mass, associated with constipation, and recently complained of pain with walking. The computed tomography-scan showed a large mass of the retroperitoneum, compressing the right ureter with upper urinary tract dilation. The alphafoetoprotein (AFP) blood level was 7500 ng/mL.

We report the case of a 2.5 years old boy diagnosed with a painless testicular mass during a clinical exam before circumcision. Testicular ultrasound revealed a vascularized 2 × 2 cm lower pole right hypoechogenic intratesticular solid lesion with calcifications, heterogeneous and distinct from the normal parenchyma. Biological markers were negative (AFP, BHCG, and LDH).

The presence of a renal agenesis with an ipsilateral seminal vesicle cyst and an ejaculatory duct obstruction is a rare congenital anomaly initially reported by Zinner in 1914. This anomaly can be responsible of perineal pain, hemospermia, hematuria, post-ejaculatory pain, urinary tract infection, infertility and lower urinary tract symptoms (LUTS).