Whipple's disease is an infectious disease caused by a gram-positive bacterium, Tropheryma whipplei. The first case was reported in 1907 by GH Whipple. Its classic symptoms are diarrhea and arthralgias, but symptoms can be various. Cardiac or central nervous system involvement, not always associated with digestive symptoms, may also be observed. For a long time, diagnosis has been based on duodenal biopsy, which is positive using periodic acid-Schiff staining. However, for patients without digestive symptoms, results can be negative, leading to a delay in diagnosis. For 10 years, a tool based on polymerase chain reaction targeting the 16S rDNA sequence has been used. In vitro culture of the bacterium, achieved 3 years ago, has allowed new perspectives for diagnosis and treatment. The natural evolution of the disease without treatment is always fatal. Current treatment is based on administration of trimethoprim-sulfamethoxazole for at least 1 year.