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PURPOSE OF REVIEW/OBJECTIVE:The goal of this paper is to review the literature on small bowel amyloidosis. Our review focuses on the underlying etiology, histopathology, clinical features, endoscopic and radiologic findings, and the mainstay of management. RECENT FINDINGS/RESULTS:The latest research shows changing epidemiological trends of different types of amyloidosis. It also reveals a better understanding of its pathophysiology and shows improvement in treatment outcomes. Amyloidosis is a group of diseases of multiple etiologies and clinical presentations. It is characterized by pathological deposition of insoluble fibrillar proteins within various organs leading to disruption of their structure and function. The classification of amyloidosis includes primary, secondary, dialysis-related, senile, and hereditary. Amyloidosis can be systemic or localized. The incidence of AA amyloidosis is declining in frequency. If the gastrointestinal (GI) tract is involved, the small intestine is the most commonly affected site. Overall, outcomes among patients with newly diagnosed amyloidosis have improved. This article focuses on small bowel amyloidosis.