Endoscopic or macroscopic esophageal melanocytosis is a benign clinicopathologic entity characterized by melanocytic proliferation in esophageal squamous epithelium and melanin deposition in the mucosa. Little is known about the etiology and natural course of this condition, although it has been suggested to be a precursor of primary esophageal melanoma by some authors. Following a search of the bibliographic databases (PubMed and Medline) regarding esophageal melanocytosis and melanosis, thirty-four cases of isolated esophageal melanocytosis (including one unpublished case from us) were found. The histopathologic features of esophageal melanocytosis are reviewed and its differential diagnosis with other pigmented esophageal lesions is discussed.