The authors made an up-to-date review of the literature concerning the management of Zinner's syndrome and evaluated a young patient with Zinner's syndrome who had presented with urinary and ejaculatory complaints. Physical examination and transrectal ultra-sonography showed a 7.0 cm right seminal vesicle cyst. Magnetic resonance imaging (MRI) confirmed the diagnosis of Zinner's syndrome. Oligoasthenoteratozoospermia was present at the two seminal analyses. Symptomatic improvement was achieved with conservative measures. Actually, the patient is still on a follow-up programme. The diagnosis is usually established at the age of increased sexual activity. Patients may be asymptomatic or present pain, irritative urinary or ejaculatory symptoms and infertility. MRI has proved to be the best imaging examination. Treatment should be adapted to symptoms, surveillance being the best option in the absence of clinical manifestations. Surgical approach may be adequate when conservative measures prove ineffective. Zinner's syndrome should be suspected if a male young patient presents with unilateral renal agenesis and pelvic complaints and has a supraprostatic mass on digital rectal examination. The initial approach should be medical, but invasive procedures may be the only way to solve the patient's complaints. Nowadays, laparoscopic and robotic techniques must replace the open surgical approach.