This is a systematic review of current evidence regarding the efficacy of cardiac resynchronization therapy (CRT) on patients with dilated cardiomyopathy and isolated left ventricular noncompaction (IVNC). This systematic review was performed following Preferred Reporting Items for Systematic Reviews and Meta-analysis guidelines. Records were searched in Pubmed, Cochrane Library, Google Scholar, Biomed Central. We included only studies focused on ventricular noncompaction patients treated with CRT. Of 46 records screened, we included 14 studies involving a total of 70 patients. All studies showed a reduction of New York Heart Association class and an increase of the ejection fraction that ranges from 8 to 36% at follow-up after CRT. Analyzing the type of response to CRT, approximately 50% of the patients were classified as responders to the therapy and most of them were super-responders. In conclusion, CRT provides beneficial effects in terms of clinical status and left ventricular function on IVNC patients with heart failure. CRT responders seem to have a great left ventricular reverse remodeling supporting the theory that CRT is able to provide an additional benefit in the IVNC disease, improving the performance of IVNC segments, when paced.