Serious ocular disease following varicella (chickenpox) is rare in children. In addition, retinitis in children with hematologic malignancies may present a difficult diagnostic challenge because infectious retinitis may mimic leukemic involvement of the eye. We report a 7-year-old patient with T-cell acute lymphoblastic leukemia in remission who presented with visual complaints 2 weeks after developing chickenpox. Ophthalmologic evaluation revealed acute retinitis in the right eye. Prolonged therapy with acyclovir resulted in near complete recovery. Early diagnosis of VZV retinopathy and aggressive antiviral treatment is critical to prevent acute and long-term ocular sequelae.