Background. West Nile virus (WNV) is an uncommon arbovirus infection and is usually asymptomatic in pediatric patients and due to its rarity is not very well known by clinicians.

Case. We present a 5-year-old girl admitted to the Pediatric Emergency Service with fever, vomiting, neck stiffness, walking difficulty and sudden deviation of eyes who was diagnosed with a neuroinvasive WNV infection.

Conclusions. Ophthalmoplegia is an unusual presentation of neuroinvasive WNV and there are no published pediatric cases with ophthalmoplegia in the literature.

Keywords: West Nile Virus, ataxia, children, ophthalmoplegia