We describe a 16-month-old girl who suffered from encephalopathy leading to intensive care unit hospitalization, complicated by Guillain-Barre syndrome and hydrocephalus, and who had serologic and molecular evidence of central nervous system infection by B. quintana. The possible association of B. quintana with Guillain-Barre syndrome and hydrocephalus has not been previously described and demonstrates the growing spectrum of neurologic complications of Bartonella spp. infections.