Saw-tooth cardiomyopathy (STC), a rare form of left ventricular cardiomyopathy characterized by saw-tooth like myocardial projections extending from the lateral walls towards the ventricular cavity, is a newly discovered cardiomyopathy first described in 2009. Detailed cardiac magnetic resonance can demonstrate multiple dense myocardial protrusions originating from the inferior wall, interventricular septum and lateral ventricular walls, which differ from typical left ventricular noncompaction. STC case reports are increasing since the first discovery. A total of ten cases have been reported. This review focuses on the clinical presentation and imaging features of this disease and analyzes the latest evidence regarding STC. Furthermore, we summarize the clinical evidence from the current decade, which may enhance detection and diagnosis of this disease in the future.