Ewing's sarcoma was first reported by J.Ewing in 1921, which is generally originated from soft tissue of the trunk or limbs. Primary Extraskeletal Ewing sarcoma (EES) of pleura is an uncommon condition, which is challenging to diagnose, and rarely reported. Herein, we present a previously 14-year-old male patient with fever and dyspnea for 1 month presented to the department of respiratory medicine in Binzhou Medical University Hospital. Radiology revealed a soft mass with massive pleural effusion in the right side of pleural cavity. After admission, we performed the transthoracic catheter drainage for the patient, followed by thoracoscopy and biopsy. Histopathology revealed a small round cell malignant tumor, combined with immunohistochemistry assay and the Fluorescence in-situ hybridization (FISH) detection of EWSR1 gene arrangement, Ewing's sarcoma was finally diagnosed. Despite receiving chemo- and radiotherapy, the patient died 1 year later after diagnosis. This paper reports a rare case that originated in parietal pleura with massive pleural effusion of Ewing's sarcoma, which was not previously reported. This rare tumor and its unusual clinical manifestations prompt us to report the current case.