Dieulafoy's lesion is a rare but potentially life-threatening cause of upper gastrointestinal (UGI) bleeding. It is characterized by an abnormally large, tortuous artery in the submucosa that can erode the overlying mucosa and cause sudden, severe hemorrhage. While commonly found in the stomach, Dieulafoy's lesions in the esophagus are extremely rare and pose a diagnostic and therapeutic challenge. We report the case of a 23-year-old female with cerebral palsy, maintained on valproate and baclofen, who presented with multiple episodes of hematemesis. Laboratory findings revealed a hemoglobin level of 4 g/dL, significantly reduced from her baseline of 12 g/dL. After resuscitation with blood transfusions, the patient underwent urgent esophagogastroduodenoscopy, which identified an actively bleeding Dieulafoy's lesion in the distal esophagus. Initial hemostatic clip placement failed, and hemostasis was successfully achieved using argon plasma coagulation (APC). The patient remained stable after the procedure and was discharged upon clinical improvement. Esophageal Dieulafoy's lesion, though rare, should be considered in severe UGI bleeding. Endoscopic hemostasis is the mainstay of treatment, and APC can be effective when other methods fail.