Esophageal melanocytosis is a rare clinical and pathological condition characterized by non-atypical melanocytic proliferation and increased melanin in the esophageal mucosa, which is normally histologically non-melanocytic. Intensive melanin accumulation and hyperpigmentation are necessary for endoscopic recognition. Due to the fact that it is a rare gastrointestinal system pathology, experience and knowledge about its diagnosis, treatment, and course are also limited. Although it is argued that chronic stimulating factors have an influence, there is no clear information about its etiology and pathogenesis. Malignant melanomas and melanocytic nevi in particular come to the fore in the differential diagnosis. Opinions and findings indicating that melanocytosis may be a precursor for malignant melanomas make the diagnosis and follow-up of this clinical and pathological condition more important. In this article, a patient with esophageal melanocytosis diagnosed by endoscopic evaluation is presented, with the aim of increasing the awareness of clinicians, especially endoscopists and pathologists, on this subject.