Lyme disease is caused by a tick-transmitted spirochete, B. burgdorferi. It can present with both central and peripheral nervous system manifestations, including aseptic meningitis, meningoencephalitis, Bell's palsy and other cranial neuropathies, radiculoneuritis, and myelitis. However, pseudotumor cerebri associated with Lyme disease is rare. Here, we report a eight-year-old girl with the unusual manifestation of pseudotumor cerebri associated Lyme disease.