Bilateral acute retinal necrosis (BARN) represents a broad ophthalmological field of severe retinal pathologies associated with poor visual prognosis and blindness. The purpose of this review is to examine the clinical spectrum in detail over the past few years, exploring laboratory and instrumental diagnosis, and providing useful and up-to-date guidance in this field. A systematic review of this field has been performed through the PRISMA guidelines, searching in the PUBMED database. Serological laboratory tests on blood or polymerase chain reaction (PCR) on aqueous humor or vitreous samples are crucial to identifying the underlying cause and choosing the timeliest strategic treatments. Often, the main cause remains herpesviruses, with Varicella zoster (VZV) predominating over the others. There are also other causes that one needs to be carefully aware of. Anatomical and functional recovery is unfavorable if they are not individuated in a quick time. Early diagnosis and timely treatment offer a better chance of visual improvement and the avoidance of complications. Complications worsen the visual prognosis over months and may require a surgical approach.