Lyme disease is reportable, and approximately 30,000 cases/year are notified to the local and state health departments. However, other estimates based on insurance records suggest there are close to 476,000 cases/year that are diagnosed and treated. In addition to a large burden of illness, areas where Lyme disease is common are expanding. Therefore, clinicians should be aware of uncommon presentations of this condition. We describe the case of a 5-year-old girl who presented with papilledema as an isolated manifestation of Lyme disease. Of note, her ocular symptoms were intermittent and worse when tired. In endemic areas, Lyme disease must be considered in the differential diagnosis for patients presenting with isolated ophthalmic findings even outside the usual Lyme season.