Primary pulmonary myxoid sarcoma (PPMS) is an extremely rare low-grade sarcoma characterized by the EWSR1-CREB1 fusion gene newly included in the latest World Health Organization classification. The patient was a 44-year-old male. Computed tomography showed a 2.1-cm-diameter solid nodule in the left superior segment (S6), and left S6 segmentectomy was performed. Histopathological examination revealed a multinodular and lobular tumor. This tumor was composed of polygonal and spindle cells within a prominent myxoid stroma. Fluorescence in situ hybridization revealed clear signal separation within the tumor, demonstrating the presence of EWSR1 gene rearrangement. Although EWSR1-CREB1 gene fusion was not confirmed by reverse transcription-polymerase chain reaction, the tumor was diagnosed as PPMS based on the typical histological findings of PPMS and presence of EWSR1 gene rearrangement.