Idiopathic ventricular fibrillation is responsible for approximately 5%-7% of cases of aborted cardiac arrest. Recent studies have shown that short-coupled ventricular premature complexes are present at the onset of idiopathic ventricular fibrillation in 6.6%-17% of patients. The present review provided information on 86 patients with short-coupled malignant ventricular arrhythmias that were reported as case reports or small patient series during the last 70 years. In 75% of the 81 cases published during the last 40 years, extended information and follow-up (from 2.63 ± 4.5 years to 10.67 ± 7.8 years; P < 0.001, between the original publication to the latest update) could be obtained from the authors. The review shows that short-coupled malignant ventricular arrhythmias occurred almost equally in males and females, at the mean age of 40 years. A tendency for later occurrence of the arrhythmia by 4 years was observed in females. A prior history of syncope was noted in 45.3% of the patients, whereas arrhythmic storm occurred in 42% at presentation. The most common mode of revelation of short-coupled malignant ventricular arrhythmias was syncope (53.5%), followed by aborted cardiac arrest (26.7%) and recurrent arrhythmic event after prior implantable-cardioverter defibrillator implantation for idiopathic ventricular fibrillation (17.4%). For the first time, short-coupled malignant arrhythmias exhibiting "not-so-short" coupling intervals (≥350 milliseconds) were found in a significant proportion of patients (17.4%). During long-term follow-up, quinidine yielded a slightly higher success rate in arrhythmia control than ablation. Larger studies are necessary to assess the best strategy for the management of this potentially lethal arrhythmia.