BackgroundThe incidence of angioimmunoblastic T-cell lymphoma is rare worldwide, and it has a poor prognosis. There is no proven or standard first-line therapy that works for the majority of patients with angioimmunoblastic T-cell lymphoma because of the rarity of this disease. The treatment and management are challenging for clinicians.Case presentationThis report presents the diagnosis and treatment of a 65-year-old Chinese man who presented with cough and lymph node swellings in the left axillary region. The patient was diagnosed with angioimmunoblastic T-cell lymphoma. He underwent eight cycles of chemotherapy with CHOP (cyclophosphamide, hydroxydaunorubicin, oncovin, prednisone) followed by TOMO radiotherapy (helical tomotherapy, a kind of radiotherapy for cancer treatment using spiral computed tomographic scanning). After treatment, the therapeutic effects were evaluated by magnetic resonance imaging and computed tomography about every 3 months. The patient recovered well with no sign of tumor recurrence and no obvious severe treatment-related adverse effects.ConclusionThis treatment experience indicates an essential role for the combination of radiation therapy with CHOP, which may have a better prognosis than treatments without radiation therapy. But challenges warrant further validation in prospective studies.