Myocardial dysplasia involves a quasi-complete or localized absence of myocardial fibers in the right or left ventricle, with corresponding partial or complete filling by fibroadipose tissue. This disorder, in its full form called “Uhl's anomaly”, was first described in 1952 as an anomaly of the heart conferring a parchment-like appearance to its walls. Incomplete forms of the disorder fall into the category of right or left arrhythmogenic ventricular dysplasia. Although ∼50 cases of right ventricular dysplasia have been documented, only two reports concern left ventricular dysplasia. Having encountered the latter anomaly, we first present the case and our observations, and then review the etiology, pathogenesis, clinical aspects, and evolution of this disorder.