Deciduoid mesothelioma is an extremely rare variant of malignant epithelioid mesothelioma. This report presents the case of a 13-year-old boy with this type of tumor in his pleura, whose initial main symptoms were chest pains and progressive scoliosis. Ensuing chemotherapy, which comprised pemetrexed and cisplatin, yielded good response after 5 cycles. Subsequent radical surgery was carried out and another 3 cycles of chemotherapy were given. The patient has been doing well 2 years after completion of this regime of treatment.