Testicular torsion (TT) occurs when the testis rotates around the axis of the spermatic cord attachments and prevents blood flow to the testis, resulting in tissue ischemia. If this occurs in the first month of life it is referred to as “perinatal TT” (PTT) or “neonatal TT” (NTT). PTT has an incidence of 6.1 per 100,000 live births. Some of these cases occur prenatally. It can be missed on the initial newborn examination, as it can be asymptomatic. Hence, the true incidence is much higher since it is underdiagnosed. The types of TT include extravaginal, intravaginal, and mesorchial. Most cases of PTT are extravaginal. The diagnosis can generally be made on physical examination. Ultrasonography (US) can help exclude other rare diagnoses as long as surgical intervention is not delayed. There has been some debate regarding the timing of surgery. Although the torsed testicle may not be salvageable, the likelihood of asymptomatic bilateral TT has to be borne in mind and contralateral orchiopexy done at the time of exploration would prevent an asynchronous torsion. Nonoperative maneuvers to detorse in PTT are not recommended. The evaluation, diagnostic approach, and management of this relatively rare condition are described.