Primary malignant melanoma of the esophagus is an extremely rare disease: among esophageal tumors its incidence is only 0.1% to 0.2%. The prognosis is generally poor due to the high potential for metastasis. Differential diagnosis of metastatic malignant melanoma can be a challenge for the group treating it. Moreover, managing it requires a multidisciplinary team which includes a gastroenterologist, a pathologist, an oncologist, a medical oncologist, and a dermatologist. We report an in situ case of primary malignant melanoma of the esophagus in a 45 -year-old patient which was managed with three esophagectomies at the National Cancer Institute of Colombia. In addition, we review the literature.