Background: The treatment of distal humerus fractures is often challenging in osteoporotic elderly patients. Total elbow arthroplasty (TEA) is a salvage option for non-reconstructable fractures. The aim of this systematic review was to evaluate the clinical evidence for primary TEA in patients with acute distal humeral fractures. Methods: Literatures were searched through PubMed, Ovid/Medline, Cochrane, Google Scholar, and Embase databases with the keywords, “distal humerus fracture,” “total elbow arthroplasty,” and “outcome” according to the MeSH (Medical Subject Headings) index for English-language studies published from April 2009 to April 2019. We performed a systematic review using Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. Results: Ten articles with a total of 269 patients were included in the review. The Bryan-Morrey approach was the most common surgical approach (33.7%) with triceps reflecting (42%) for triceps tendon management. The most common implant design used was the Coonrad-Morrey system (83%). The mean postoperative motion arc was 102.3° for flexion-extension and 145.8° for pronation-supination. The average functional outcome score was 89.5 with Mayo Elbow Performance Score (MEPS). An excellent MEPS was found in studies with less than 7 days of average time from injury to surgery. The overall complication rate was 21.5%. Conclusions The current review showed favorable outcome of primary linked TEA for acute distal humerus fractures. Despite the promising functional outcomes, the complication rate was still considerably high. This systematic review will give surgeons help in explaining to patients regarding the expected outcome after primary TEA for acute distal humerus fractures.