Celiac disease is an immune mediated enteropathy in susceptible individuals as a response to gluten containing diets based on wheat, oat, rye and maize. It clinically presents with malabsorption syndrome along with a myriad of extraintestinal manifestations such as anemia, osteoporosis, dermatitis herpetiformis, peripheral neuropathies, ataxia and cognitive impairment. Although the prevalence of these extraintestinal features range from 1 to 15% in these patients, their presence in the absence of intestinal manifestations is very rare. Here we report the case of a middle aged female diagnosed with celiac disease with coexisting gluten sensitive ataxia in the absence of gastrointestinal symptoms.