Background: Polyorchidism is a rare congenital anomaly of the urogenital tract. Usually, it requires conservative management; however, this varies according to each patient. This report summarizes the possible clinical presentation and the feasible management options depending on the patients and testicular characteristics. Case presentation: 23 years old young male presented to our outpatient clinic with intermittent left testicular pain associated with tenderness to palpation. On the physical exam, he had a palpable mass at the upper pole of the left testis. He was taken to an MRI, which showed a normal right testis with a left mass-dependent of the upper pole of the left testis. He was discharged after pain control was achieved; however, he presented again to the Emergency Room with acute left testicular pain one week later. The physical exam was suggestive of left testicular torsion. He was taken to surgery for scrotal exploration and bilateral testicular fixation. Polyorchidism was confirmed intraoperatively. Conclusions: Polyorchidism is a rare pathology of the male genitalia. When suspected, a thorough physical examination is always mandatory. It is essential to individualize the patients’ cases to decide whether conservative management is attainable, with radiologic studies support.