Introduction: Dieulafoy's lesion (DL) presented with small bowel bleeding constitutes a group of rare and potentially life-threatening prognosis. Several case series have described this condition, yet it remains unclear as to what is the optimal treatment and predicted outcome for patients who have been diagnosed. Patient concerns: We present a 21-year-old male experiencing bloody stool for 1 day. Diagnosis: Computed tomography of the abdomen exhibited active contrast extravasations and segmental wall thickening in the jejunum, and enteroscopy showed one 15-millimeter sized subepithelial tumor at the proximal jejunum. Interventions: Due to unstable vital signs he received an emergent transcatheter arterial embolization, and surgeon performed a laparoscopic surgical resection thereafter under the impression of potential malignancy. The pathologist confirmed jejunal DL with organizing thrombus. Outcomes: He was discharged on the 8th day of hospitalization without recurrent bleeding. Conclusion: A systematic literature review of 98 published cases taken from PubMed dating back to 1978 was undertaken, and the patients with DL and small bowel bleeding involved mainly the jejunum, followed by the duodenum and ileum. Meanwhile, DL-related duodenal bleeding was diagnosed mostly by an enteroscopy, as well as endoscopic interventions. Jejunal and ileal bleeding due to DL was surveyed through endoscopy and surgery, while surgical resection remained the choice for bleeding cessation. Only anticoagulant use (OR = 18.16; P = .08) was associated with a higher risk of overall mortality, although it was non-significant in univariate analysis. We emphasize that individualized treatment as well as prompt measurement should be implemented accordingly.