Amyloidosis is characterized by the extracellular deposition of an abnormal fibrillar protein, which disrupts tissue structure and function. Amyloid may be localized to a single organ, such as the GI tract, or be systemic where the amyloid type is defined by the respective fibril precursor protein. Among patients with systemic amyloidosis, histological involvement of the gastrointestinal (GI) tract is very common but often subclinical. The presence and pattern of GI symptoms varies substantially, not only between the different amyloid types but also within them. GI presentations are frequently nonspecific and include macroglossia, dyspepsia, hemorrhage, a change in bowel habit and malabsorption. Endoscopic and radiological features of amyloidosis are also nonspecific, with the small intestine most commonly affected. In the absence of specific treatments for GI amyloidosis, therapy is aimed at reducing or eliminating the supply of the respective fibril precursor protein. Supportive measures such as nutritional support and antidiarrheal agents should be instigated while awaiting the clinical improvement associated with a successful reduction in the abundance of the fibril precursor protein. GI tract surgery should be performed only if the benefits clearly outweigh the risks, as there is a risk of decompensation of organs affected by amyloid.