A T>G single nucleotide polymorphism (SNP, rs2279744) of the MDM2 gene has been investigated in sarcoma community, but the findings are conflicting. This study was designed to well define the relationship between SNP rs2279744 and sarcoma risk. We did a systematic computerized search of the PubMed, Web of Science, and Science Direct databases to identify the human case-control studies investigating the relationship between SNP rs2279744 and sarcoma risk with complete genetic data. Pooled odds ratios (ORs) were calculated with the Mantel-Haenszel fixed-effect model or the DerSimonian and Laird random effects model to estimate the risk of sarcoma. Overall analysis included five independent studies. On the whole, the T/G genotype or the combined G/G and T/G genotypes appeared to be associated with approximately 1.40-fold higher risk of sarcoma relative to the T/T genotype (T/G vs. T/T: OR 1.33, 95% CI 1.00-1.77; G/G + T/G vs. T/T: OR 1.42, 95% CI 1.08-1.85). We noted that the Caucasian populations showed a similarly increased risk of sarcoma ascribed to the carriage of the same genotypes (T/G vs. T/T: OR 1.41, 95% CI 1.05-1.90; G/G + T/G vs. T/T: OR 1.49, 95% CI 1.13-1.97). This meta-analysis provides evidence that MDM2 SNP rs2279744 may be significantly associated with increased risk of sarcoma in Caucasian individuals.