We reported a rare case of intraocular post-transplant lymphoproliferative disorder (PTLD) arising in a 3-year-old liver transplant recipient who had a prior history of systemic PTLD. The first PTLD entered remission after treatment with intravenous rituximab and withdrawal of immunosuppressants. One year after remission, she presented with granulomatous uveitis and iris nodules in the right eye. Iris biopsy confirmed recurrence of intraocular PTLD, which resolved completely after a second course of intravenous rituximab. Pediatr Blood Cancer 2015;62:169–172. © 2014 Wiley Periodicals, Inc.