Zinner syndrome (ZS) is a rare anomaly of the Wolffian duct. We searched for case reports and case series to identify the most effective methods for examination and treatment. The PubMed/Medline, Embase, and Scopus databases were searched. Our searches yielded 160 case reports/case series and included 214 patients. The mean age at diagnosis was 29.35 years. The R:L distribution was 1:1. The most common symptoms were: frequency (24.3%), dysuria (23.1%) and perineal pain (20.2%) in ZS patients with clinical symptoms. The diagnostic investigations used most frequently were magnetic resonance imaging (MRI) (67.8%) and ultrasonography (65.0%). Maximum flow rate on uroflowmetry, semen ejaculate volume, sperm count, and sperm motility were significantly lower in patients with ZS. Among 193 patients with treatment details, 65.8% underwent surgery; 9.8% underwent aspiration; 24.3% were followed with observation. Two patients died after surgery; all other patients remained asymptomatic after surgery. Fifty-two patients had complications and comorbidities. In patients with ZS, the most common symptoms are seminal vesicle cyst enlargement and compression of the bladder, ureter, and reproductive system. The diagnosis mostly depends on radiological examination. Surgery may be effective, but complications may occur.