Synovial sarcoma is a rare cancer which occurs primarily in the extremities of young adults. Among them, synovial sarcoma of pleura is a very rare type. Only less than 50 cases have been reported in the literature. Here we report a unique case of synovial sarcoma of pleural. The patient was a 53-year-old man complained of cough, hemoptysis and shortness of breath for 5 month. Chest CT scan revealed a large mass lesion in the left lung, and several metastatic legions in both lungs. No other organs were involved at the time of examination. The patient had synovial sarcoma at right plantar nine years ago. The tumor was surgically removed and followed with radiotherapy for half a month. The patient did regularly follow-up examinations for 5 years, and no metastasis and recurrence were found. He was in remission for 9 years. The patient was admitted in hospital, and the fine needle biopsy and histology studies supported the diagnosis of primary synovial sarcoma of pleural. Cytogenetic fluorescence in situ hybridization (FISH) confirmed the tumor had t(X;18) chromosomal translocation. He received 2 cycles of chemotherapy, and unfortunately tumor didn't response to the treatment. The patient was discharged without further treatment. This is the first case report that patient developed a second primary synovial sarcoma of pleural after nine years remission of the first primary synovial sarcoma in plantar. IJCEP Copyright © 2019.