Acute cerebellar ataxia and opsomyoclonus are presenting signs of occult neuroblastoma for a substantial proportion of paediatric patients. Cerebellar ataxia may be due to antibodies against the neuroblastoma cross-reacting with cerebellar tissue. This report is of a 26-month-old boy who presented with encephalitis-like features of ataxia, seizures, decreased consciousness, and involuntary movements. Magnetic resonance imaging of the brain and spine were normal 2 weeks after presentation. The child did not have the classical signs of opsoclonus or myoclonus at any stage of the disease but was found to have occult neuroblastoma. The late demyelinating changes seen on magnetic resonance imaging of the brain support an immunological basis for the paraneoplastic manifestations of occult neuroblastoma in this child. Occult neuroblastoma should be considered as one of the differential diagnoses for children presenting with persisting encephalitis-like features in the presence of normal neuroimaging findings.