BACKGROUND Rectal Dieulafoy's lesions (DLs) are very rare; however, they can be life threatening when presented with massive hemorrhage. CASE SUMMARY A 44-year-old female with medical history of chronic renal failure who was on renal replacement therapy presented with lower gastrointestinal hemorrhage. Physical examination revealed signs of hypovolemic shock and massive rectal bleeding. Complete blood count revealed abrupt decrease in hematocrit. After hemodynamic stabilization, an urgent colonoscopy was performed. A rectal DL was diagnosed, and it was successfully treated with two hemoclips. There were no signs of recurrent bleeding at thirty days of follow-up. CONCLUSION Rectal DLs represent an unusual cause of lower gastrointestinal bleeding. Massive hemorrhage can increase the morbidity and mortality of these patients. Endoscopic management continues to be the reference standard in the diagnosis and therapy of these lesions. Thermal, mechanical (hemoclip or band ligation), or combination therapy (adrenaline injection combined with thermal or mechanical therapy) should be considered the first choice for treatment.