Primary vitreoretinal lymphoma (PVRL), also known as intraocular lymphoma, is a rare and aggressive form of non-Hodgkin's lymphoma that primarily affects the eyes. This type of lymphoma typically involves the vitreous humor, a gel-like substance that fills the eye, and the retina, the light-sensitive tissue at the back of the eye. PVRL often presents with nonspecific symptoms, making its diagnosis challenging. Patients may experience blurred vision, floaters, and, in some cases, eye pain. The disease is more common in older adults, particularly those over the age of 50, and has a predilection for individuals with compromised immune systems. Diagnosis of PVRL involves a comprehensive eye examination, imaging studies, and analysis of cerebrospinal fluid. Ophthalmic examination may reveal characteristic findings such as vitreous opacities, retinal lesions, and subretinal infiltrates. Due to the rarity of PVRL, treatment strategies are not standardized. However, a combination of systemic chemotherapy, intravitreal chemotherapy, and radiation therapy may be employed to target both ocular and systemic involvement. Prognosis varies, and the disease can be associated with a high risk of recurrence. Given the complexity of PVRL and the need for a multidisciplinary approach, patients are often managed collaboratively by ophthalmologists, hematologists, and oncologists. Ongoing research aims to enhance our understanding of the disease and improve treatment outcomes for affected individuals. As PVRL remains a challenging condition, early detection and prompt intervention are crucial in optimizing patient outcomes.