Abstract:[Objective] To investigate the effect of long noncoding RNA 02126 (lncRNA02126) on the prognosis of Ewing''s sarcoma and its potential mechanism. [Methods] The gene expression data of 56 Ewing''s sarcoma patients were obtained from ICGC (International Cancer Genome Consortium) database. Kaplan-Meier survival analysis and univariate analysis were used to explore the relationship between lnc02126 and prognosis of Ewing''s sarcoma. RT-qPCR was used to detect the expression of lnc02126 in Ewing''s sarcoma cells. Go and KEGG analysis were used to understand the possible signal pathway of lnc02126 in the biological progress of Ewing''s sarcoma. CMAP (connectivity map) predicted the potential therapeutic drugs for Ewing''s sarcoma. [Results] The prognosis of Ewing''s sarcoma patients with high expression of lnc02126 was significantly poor (P = 0.025). lnc02126 was an independent prognostic risk factor (P < 0.001). RT-qPCR results showed that lnc02126 was highly expressed in Ewing sarcoma cells (P < 0.05). Go and KEGG analysis showed that the molecular mechanism of malignant progression of Ewing''s sarcoma induced by lnc02126 was related to the signal pathways of extracellular structure, tissue construction, cell adhesion and angiogenesis. CMap analysis showed that lycorine, iloprost and thapsigargin had potential therapeutic effects on Ewing''s sarcoma. [Conclusions] lnc02126 is an independent prognostic molecular marker of Ewing''s sarcoma and may be a therapeutic target for Ewing''s sarcoma.