This case report presents the first simultaneous occurrence of Chiari malformation type 1 and celiac disease in a 27-year-old male patient. To the best of our knowledge, there are no previously published cases in the literature. This rare association suggests that nutrient malabsorption caused by celiac disease may exacerbate the symptomatic severity of Chiari malformation. We recommend screening for celiac disease in patients diagnosed with Chiari malformation who present with anaemia, vitamin deficiency or unexplained gastrointestinal complaints. Conversely, in the presence of atypical neurological symptoms in celiac disease, craniocervical magnetic resonance imaging should be performed and a multidisciplinary approach strategy should be considered in treatment.