Background/aims: Dieulafoy's lesion of the anal canal is a very rare clinical case. Although it was first described in the stomach, there has been an increasing frequency, especially in the last decade, of reports of the lesion in the colorectal region. Methods: Herein, we report one case presenting with massive hematochezia requiring multiple blood transfusions due to a Dieulafoy's lesion in the anal canal. To our knowledge, this is the fourth case in Medline. Surgical oversewing was attempted twice but rebleeding occurred, and local excision through the anal canal was performed. Results: The patient was treated successfully with mucosectomy including the lesion. Conclusions: Sclerotherapy, alcohol and epinephrine injection, thermocoagulation and selective arterial embolization are the options of therapeutic endoscopy and interventional radiology. As for surgical management, oversewing is an alternative technique. However, in our opinion, because of the recurrent and life-threatening manner of this arterial bleeding pattern, local excision, if possible, is the most reliable management of the disease.