Dieulafoy's lesion is an abnormally large, tortuous, submucosal vessel that erodes the overlying mucosa, without primary ulceration or erosion. Although these lesions predominantly involve the stomach and upper small intestine, they are being detected with increasing frequency in the rectum. We conducted a systematic literature search of MEDLINE, Cochrane, Embase, and Scopus databases for adult rectal Dieulafoy's lesion. After careful review of the search results, a total of 101 cases were identified. The data on patient characteristics, clinical features, colonoscopy findings, diagnosis, treatment, and clinical outcomes were collected and analyzed. The mean age of presentation was 66±17 years (range, 18-94 years), with 54% of cases reported in males. Clinical presentation was dominated by acute lower gastrointestinal bleeding in the form of bright-red blood per rectum 47% and hematochezia 36%, whereas 16% of patients were admitted with symptoms related to other medical conditions. Major underlying disorders were hypertension 29%, diabetes mellitus 21%, and chronic kidney disease 16%. The average number of colonoscopies required for the diagnosis of rectal Dieulafoy's lesion was 1.5±0.7. In regard to treatment, endoscopic therapy was applied in 80%, direct surgical suturing in 12%, angiographic embolization in 4%, and endoscopic therapy followed by surgical ligation was performed in 4% of patients. The endoscopic treatment was a feasible choice for rectal disease, with a primary hemostasis rate of 88%. Although the overall mortality rate was 6%, the causes of death were unrelated to this entity. This review illustrates that patients with rectal Dieulafoy's lesion can have a favorable clinical outcome. Prompt diagnosis and appropriate management are of paramount importance to prevent serious hemodynamic complications. The best therapeutic modality remains to be determined but the data presented here support the use of mechanical endoscopic methods as safe and effective.