Malignant fibrous histiocytoma (MFH) of the lung is rare, accounting for less than 0.2% of all pulmonary neoplasms, and an optimal treatment strategy has not yet been elucidated. We encountered a 62-year-old male patient with MFH of the lung who underwent a resection of the lymph node of recurrence 7 months after a lobectomy. We herein review 93 cases of pulmonary MFH in the literature, and discuss the surgical treatment for this disorder with special reference to lymph node involvement. Lymphogenic metastasis was observed in 14 of 72 cases (19%) who underwent surgical treatment. The 5-year survival rates for these reported patients with and without surgical treatment were 43% and 0% (P = 0.01) and the 5-year survival rates for “resected” patients with and without lymph node metastasis were 27% and 49%, respectively (P = 0.03). A complete resection with the systematic dissection of regional lymph nodes is therefore considered to positively contribute to the survival of patients with primary pulmonary MFH.