Introduction: Mitral valve repair (MVr) is the gold standard for the treatment of degenerative mitral valve regurgitation (MR). The recently introduced NeoChord DS1000 has gained increasing recognition as a feasible, potentially safe, and effective procedure with minor complications and promising outcomes. This study aims to conduct a systematic review of the published literature that discusses the technical feasibility and outcome of transapical off-pump MVr with NeoChord DS1000 device implantation in the treatment of degenerative MR. Methods: This review was performed according to the PRISMA statement. Databases searched in this review included Pubmed, Web of Science, Scopus, and Cochrane databases for systematic reviews. All English articles on humans reporting isolated MVr using NeoChord DS1000 device were included provided that basic preoperative data, operative specifications, and postoperative mortality and morbidity were reported. Results: This review included six studies comprised 249 patients who had NeoChord mitral procedure. Almost all patients included had severe MR (243/249, 97.6%). Operative success was achieved in 241 out of the 249 patients (96.8%). No intraoperative mortality was reported. Intraoperative arrhythmia was reported in six patients (2.4%) and significant bleeding was reported in eight patients (3.2%). Conclusion: Awaiting more evidence, NeoChord mitral procedure appears to be a promising procedure that can be considered in selected cases.