Lyme disease is a multisystem infectious disease caused by the tick-borne spirochete Borrelia burgdorferi. CNS involvement occurs frequently. Meningitis, multiple cranial nerve palsies, neurophaties, radiculophaties, and encefalophaties are particularly common. The diagnosis should be done carefully; epidemiology, clinical features, and laboratory findings are the basis in the management of disease. In this paper we present a 12 year old Ecuadorian boy who fulfills diagnosis parameters for Lyme disease according to the American Academy of Neurology. This is the first reported case of this disease in Ecuador. It is necessary to carry out epidemiological studies to confirm the occurrence of this condition, and the look for the presence of the transmitting vector and its causal agent in our country.