Literature to date reported only 6 instances of primary pulmonary ASPS.[5–10] Due to financial and technical considerations, only 2 of the 6 cases confirmed the presence of ASPSCR1–TFE3 fusion.[6,7] This study reported a 27-year-old male case of primary pulmonary ASPS with confirmed ASPSCR1–TFE3 fusion and reviewed the currently published literature. This evidence concerns the gene ASPSCR1 and alveolar soft part sarcoma.