Primary esophageal natural killer (NK)/T-cell lymphoma is a markedly rare tumor. There were only 6 cases of the disease identified prior to June 2015. In the present study, the aforementioned cases were validated, and relevant computed tomography (CT) results and clinical features of primary esophageal NK/T-cell lymphoma were determined, to increase awareness of this type of tumor. CT features and clinical presentations of a patient with pathologically confirmed esophageal NK/T-cell lymphoma was analyzed. The patient exhibited non-specific clinical symptoms and CT images revealed diffuse thickening of the entire length of the esophagus. The patient received cycles of systemic chemotherapy and subsequent chest CT images demonstrated prompt and marked shrinkage of the tumor. At the time of writing, the patient has survived for 24 months and experiences a good quality of life without postprandial fullness or difficulty swallowing solid food. In addition, the characteristics of 6 patients with complete clinical features of this type of tumor, on the basis of a review of published studies (online PubMed, Medline, Google Scholar, Chinese Biomedicine Database and China Journal Full Text Database search), were retrospectively analyzed. Although primary esophageal NK/T-cell lymphoma is a markedly rare tumor, it is considered to be included in differential diagnosis of patients presenting with a fungal or viral infection, therapy-related mucositis or reflux esophagitis. The final diagnosis of primary esophageal NK/T-cell lymphoma is on the basis of a combination of clinical, CT and histopathological results.