A toddler presented with a 6 week history of progressively worsening ataxia, weight loss and loose stool. There was no developmental regression, history of fever or foreign travel. Extensive investigation, including cerebrospinal fluid studies and neuroimaging, were inconclusive. In coeliac screen, the endomysial antibodies were positive with above tenfold elevation of IgA TTG from normal limit, making the diagnosis of coeliac disease (CD). The association of CD with neurological symptoms in our case led to the clinical impression of gluten ataxia. She was started on gluten free diet and had complete symptom resolution in over 4 weeks' time since the start of gluten-free diet. Although common in adults, it has not been reported in toddlers, expanding the differential diagnosis of ataxia.