Purpose: To introduce a case of primary esophageal CD30-positive ALK-positive anaplastic large cell lymphoma (ALCL) and discuss its diagnosis and treatment.Methods: Esophagectomy was done for a 37-year-old male with a submucosal lesion after a frozen section failed to give a definite diagnosis. Samples were sent for hematoxylin-eosin staining and immunohistochemical analysis. Six cycles of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy were given and the patient was followed up.Results: The operation was uneventful. Postoperative pathologic and immunohistochemical examination yielded a diagnosis of primary CD30-positive and ALK-positive ALCL of the esophagus. The patient was in complete remission at the 14-month follow-up.Conclusions: ALCL of the esophagus should be considered in the differential diagnosis of esophageal submucosal lesions. Biopsy through either esophagoscopy or surgical exploration, chemotherapy, and radiotherapy can be chosen for long-term survival.