In 1994 a 19-year-old woman presented with a few weeks history of back ache. Routine chest X-ray and CT examination revealed a lesion originating from the parietal pleura and destroying the ribs. The tumour was resected during thoracotomy. The histological examination raised the possibility of atypical carcinoid tumour. One year later the tumour recurred. After its re-resection, the patient received radiotherapy. Three years after the initial presentation multiple pulmonary metastases developed. The patient was treated with chemotherapy, receiving vincristine, epi-adriamycin and cyclophosphamide in 8 cycles, which resulted in complete remission. Between 1998 and 1999 progressions and partial remissions were observed, while the patient received further cycles of chemotherapy. Histological revision was performed in 1999 and a final diagnosis of desmoplastic small round cell tumour of the pleura was made. Immunohistochemically co-expression of cytokeratin, vimentin, desmin, and NSE was observed. The patient died in June 2000. The whole follow-up period was 76 months. We thought this case to be worth for presentation because this unusual long survival, which was probably due to the aggressive complex anticancer treatment.