Whipple's disease is a rare systemic disease. It is caused by a gram-positive infectious bacillus called Tropheryma whipplei (T. whipplei). The common presenting feature is malabsorption, but it can also affect multiple organs as heart, central nervous system (CNS), joints, skin, lungs and vascular system. Presentation can be highly variable but mainly the patients are complaining of weight loss, diarrhea, joint pain and arthritis, about 15% of patients do not develop these classic symptoms. Endoscopic picture usually shows pale yellow shaggy mucosa with erythematous eroded patches in the duodenum extending up to the jejunum. Definitive diagnosis is made mainly by endoscopic duodenal biopsies that show characteristic foamy macrophages in the lamina propria containing non-acid fast gram positive bacilli that stain red with Periodic acid-Schiff (PAS) stain. Immunohistochemical staining for antibodies against T. whipplei had been used to detect the organism in a variety of tissues. Polymerase chain reaction (PCR) of T. whipplei is considered a confirmatory test. We present a case of classic Whipple's disease with review of literature for better understanding of the presentation, methods of diagnosis and treatment of the disease.