Nonceliac wheat sensitivity is a recently characterized disorder. Pathogenesis is still unclear, but it is known that not only gluten but also other wheat components, such as fermentable oligosaccharides, disaccharides, monosaccharides, and polyols and amylase-trypsin inhibitors, could activate patient’s immunity and cause the onset of symptoms. This review illustrates the progress made over the last few decades and clinical/histologic features, as well as controversies. The lack of a biomarker makes the diagnosis still difficult. It is currently a diagnosis of exclusion, confirmed by a double-blind challenge with wheat. Further studies are needed to improve the diagnostic and therapeutic approach to this disease.