A 14-month-old female infant with chronic diarrhea, recurrent respiratory infections and stunted growth was diagnosed as celiac disease with AGA detection and a positive intestinal biopsy. A gluten-free diet was introduced with a poor response. A sweat test was positive and heterozygosis for mutation of CFTR gene (both F508 and G542X) was found, demonstrating an association in the infant between cystic fibrosis and celiac disease. Fifteen cases of such association have been previously described in literature, but only three have been genetically studied. The co-existence of cystic fibrosis and celiac disease in the same subject has to be considered till now a casual finding, but are also discussed hypothesis of a non-casual linkage, formulated by some authors.