Primary central nervous system lymphoma (PCNSL) is a diffuse, large B-cell lymphoma affecting the brain, spinal cord, leptomeninges, or eyes. A patient with a recurrence of a previous PCNSL manifesting as an isolated vitreoretinal disease without central nervous system (CNS) involvement and a second cerebral recurrence without vitreoretinal involvement has not yet been reported. The patient is an 86-year-old man with PCNSL of the left cerebellum diagnosed at the age of 82 years and treated with suboccipital trepanation and resection of the lesion followed by chemotherapy. At the age of 84, he suffered a first recurrence, which manifested as vitreoretinal lymphoma on the right and later also on the left eye, without CNS involvement. The recurrence was treated by vitrectomy on both sides. At the age of 86, the patient suffered a second recurrence, which manifested as infratentorial lesions treated by radiotherapy. He was discharged with dexamethasone, entecavir, valacyclovir, amphotericin-B, candesartan, tramadol, trazodone, and pantoprazole. This case demonstrates that PCNSL can recur with isolated secondary ophthalmologic lymphoma and a second time with isolated cerebral lymphoma. Although glucocorticoids, chemotherapy, and radiotherapy can be highly effective, the long-term outcomes of patients with PCNSL are not as favorable.