Abstract Aims Myocardial calcification is an unusual condition in which excess calcium is deposited in the myocardium. We herein report two cases of myocardial calcification from our clinical experience. Furthermore, we conducted a systematic review to examine the clinical course and associated pathologies of myocardial calcification. Methods and Results This systematic review was registered in PROSPERO (CRD42023463285). PubMed and Scopus were searched according to the following inclusion criteria: (a) case reports or case series describing patients with myocardial calcification; (b) diagnosis of myocardial calcification by computed tomography; (c) adequate description of patients, including their chief complaint, medical history, evaluations, treatments; and (d) publication in English. Among the 75 patients, 24 had sepsis, 14 had myocarditis, and 37 had other pathologies. The mortality rate was 33% for patients with sepsis, 14% for patients with myocarditis, and 11% for patients with other pathologies. Follow up computed tomography findings beyond 2 years were reported in six patients, showing that the computed tomography findings of myocardial calcification persisted but subsided over time. Autopsy was performed in seven patients, and extensive interstitial fibrosis and collection of inflammatory cells were observed in patients with myocarditis, sepsis, and ischemic heart disease. Conclusion While various medical conditions can cause myocardial calcification, accompanying conditions commonly reported with myocardial calcification were sepsis and myocarditis. The computed tomography findings of myocardial calcification tends to regress over time, if the underlying disease can be treated.