Duodenal biopsy is an essential component in the diagnosis of celiac disease (CD). Although the classical findings of increased number of intraepithelial lymphocytes, crypt hyperplasia and villous atrophy are very characteristic, the diagnosis cannot be achieved on the basis of histopathology alone, as there are many entities that can mimic CD and a close collaboration of a pathologist and a clinician specialist is needed. In a patient with suspected CD, pathologist should describe essential histopathological findings and offer differential diagnosis when appropriate. The most important recent changes in the diagnostic criteria for CD include lower numbers of intraepithelial lymphocytes that are considered to be normal and recommendation to perform biopsies from the proximal part of the duodenum in addition to the distal duodenal biopsies.