Congenital aneurysms of the left ventricle (ALV) are rare cardiac lesions. Beyond that an association with malignant ventricular arrhythmias (MVA, symptomatic ventricular tachycardia--VT or ventricular fibrillation--VF) is reported only in sporadic cases. Since 1988 we had the opportunity to study 5 patients (pts) with MVA (4 sustained VT, 1 VF; 1 female, 4 males; mean age 38 years) without cardiovascular risk factors, history of myocardial infarction, trauma or inflammatory disease. Left ventricular contrast angiography and echocardiography disclosed ALV's. At programmed electrical stimulation clinically documented MVA (4 VT, 1 resuscitated VF) were reproducible in all 5 cases, the respective VT was located in the area of the ALV in 4 cases. In 2 pts aneurysmectomy combined with subendocardial resection and cryotherapy (1 apical, 1 posterobasal ALV) was performed. In both pts histopathology confirmed a congenital disorder, without evidence of inflammatory lesions. In 2 pts MVA was controlled with antiarrhythmic therapy. The pt with VF and an ALV adjacent to the anulus of the aortic valve received an implantable cardioverter defibrillator. In congenital aneurysms of the left ventricle complicated by malignant ventricular arrhythmias surgical intervention offers a potential cure in selected cases.