Spontaneous acute esophageal necrosis (AEN) has been reported to be extremely rare. The condition is defined as a dark pigmentation of the esophagus associated with histologic mucosal necrosis. The exact pathogenesis is still unknown, but several etiologies have been suggested including ischemia, gastric outlet obstruction, hypersensitivity to antibiotics, gastric volvulus and viral infection. We herein present a case of a middle-aged man with end-stage renal disease who presented with AEN following a hemodialysis session. Its diagnosis and management are discussed with reference to the pathogenesis of the condition.