Collagenous sprue (CS) was once thought to be closely related to celiac disease (CD) due to the similarities in histology such as flattened mucosal villi with biopsy, and clinical features such as hyposplenism, positive endomysial antibodies and the subsequent development of T-cell and B-cell lymphomas (1). However, there are significant differences between CD and CS, which make them two pathologically distinct entities. CS is characterized by the complete atrophy of the