Malignant fibrous histiocytoma (MFH) is rare in the chest wall, particularly in patients who have undergone radiotherapy for primary nasopharyngeal cancer. In the present study, a case of MFH of the upper chest wall that appeared four years after initial radiotherapy for squamous cell carcinoma of the nasopharynx is reported. Furthermore, two-step surgical management was successfully performed consisting of i) tumor-reductive excision and ii) limb salvage surgery, including wide resection of the tumor mass, defect reconstruction of the chest wall using left latissimus dorsi myocutaneous flap and dermatoplasty of the flap-supplied region. The progress of the clinical characteristics, the reasons for radiation-induced carcinogenesis, the treatment options and the prognostic factors of MFH are also reviewed. Finally, the importance of prevention and follow-up of this malignancy are highlighted and specific advice is offered.