Chronic nonspecific multiple ulcers of the small intestine is a rare but distinct clinical condition, characterized by multiple small intestinal ulcers of nonspecific histology and chronic, persistent gastrointestinal bleeding without nonsteroidal anti-inflammatory drug use. However, because of the term "nonspecific" in its nomenclature, some gastroenterologists have misinterpreted the disease as the condition with small intestinal ulcers caused by undetermined etiologies without considering clinical features. Such misinterpretation has led to the heterogeneity of clinicopathologic features of the disease, as well as to ambiguity regarding a possible genetic contribution. It thus seems necessary to recognize the clinical entity of the disease precisely to avoid misinterpretation. In this review, we describe the clinicopathologic features, differential diagnosis, and the possibility of a genetic contribution to the disease.