Myxoid liposarcoma (MLPS) is genetically characterized by FUS-DDIT3 or EWSR1-DDIT3 gene fusion and high frequency of hotspot mutations (C228T or C250T) in the promoter region of TERT which encodes telomerase reverse transcriptase (TERT). The latter leads to telomerase reactivation, a mechanism of telomere maintenance. Although TERT promoter hotspot mutation is a poor prognostic factor in various tumors, its effect on MLPS has not been reported in detail. In the present study, we examined the clinicopathological characteristics, prognosis, and telomere maintenance mechanisms in 83 primary tumor samples of MLPS, which was resected surgically at Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan, from 2008 to 2020. TERT promoter hotspot mutations were observed in 77% (63/82) cases, and alternative lengthening of telomeres (ALT) was absent in all cases. Among the cases without TERT promoter hotspot mutations, TERT rearrangements and minor point mutations in the TERT promoter region were found in three and two cases, respectively. TERT mRNA expression was consistently observed even in cases where no genomic TERT aberrations were detected, and the presence of TERT promoter hotspot mutation did not correlate significantly with either overall and metastasis-free survival (p = 0.56, p = 0.83, respectively) or clinicopathological features. Thus, MLPS characteristically shows TERT expression and high prevalence of TERT aberrations. Our findings suggest that TERT aberration is not prognostic factor, but might occur at an early stage and play a key role in tumorigenesis.