Introduction/Background: Kinsbourne syndrome or opsoclonus mioclonus ataxia is a rare syndrome, commonly post viral or paraneoplastic, characterized by opsoclonus, ataxia, myoclonic jerks of trunk and limbs and behavioral disturbances. Affects both adult and pediatric ages, with greater prevalence between one and three-year-old and equal gender prevalence. Neuroblastoma, hepatoblastoma and glangioneuromas are ordinarily associated with this syndrome. This paperwork aims to present a case report concerning Kinsbourne Syndrome and review literature about it. Case presentation: We report a case of a one-year-and-two-month-old male, with opsoclonus, gait disturbance and imbalance associated with a neuroblastoma, who has undergone therapy with prednisone 1mg/kg/day and surgical resection, evolving with complete resolution of symptoms after four years and a half of clinical following, presenting himself to the date without neurological deficits, contrary to literature statistics. Conclusion: KS is ordinarily a post-viral or paraneoplastic rare syndrome, with higher prevalence between 1 to 3-year-old infants, nevertheless also affecting adults. araneoplastic syndrome is majorly associated with NB