Lyme disease is a multisystemic disorder caused by Borrelia burgdorferi spirochete, transmitted by ticks. Mainly described in the northern hemisphere and rarely in Brazil. The purpose of this report is to describe a patient with Lyme disease who developed bilateral tonic pupil as the only remaining sign. A 13-year-old female with Lyme disease, presented with bilateral peripheral facial and oculomotor paralysis. After recovery from neurological abnormalities the patient sustained anisocoria, reduced fotomotor reflex, less than normal amplitude of accommodation, tonic pupil constriction for near objects and slow redialation in both eyes. Dilute 0.1% pilocarpine test was positive in both eyes, confirming the suspicion of bilateral tonic pupil. This is the first case report of bilateral tonic pupil caused by Lyme disease.