To present a pediatric patient with a subacute progressive cerebellar syndrome associated with anti-Tr/Notch-like epidermal growth factor-related receptor (DNER) antibody highlighting the need for optimizing treatment in pediatric patients.

Anti-Tr/DNER-associated cerebellar ataxia is a rare disorder, predominantly affecting middle-aged males and highly associated with Hodgkin’s lymphoma. Patients typically present with cerebellar ataxia prior to tumor diagnosis. Due to the paucity of literature surrounding pediatric cases, optimal treatment remains unclear as current recommendations are based on a systemic case review spanning a wide age range with a mean age of 47.

We present a pediatric case of anti-Tr/DNER-associated cerebellar ataxia.  While commonly associated with malignancy, none was found in the patient to date.  Patients’ responses to immunotherapy can be poor, although prior analysis suggests that younger patients have a higher prevalence of favorable neurological outcomes. Currently there is no standard approach to treatment, however we report a favorable response to first line therapy with steroids and IVIg, followed by second-line therapy in this case.