Congenital isolated left ventricular (LV) diverticulum is a rare disease and has only been documented by a few case reports and a small series of retrospective analyses. Presented is a 22-year old male patient, previously well and with no risk factors for coronary heart disease, who complained of a one month history of chest heaviness and easy fatigability, physical findings revealed a dynamic precordium and a grade 3/6 mid systolic murmur at 5th and apical areas. Chest radiography showed a large convex density in the left side of the cardiac silhouette and interstitial and alveolar edema in the right middle and lower lung. Both transthoracic and transesophageal echocardiography showed a non-contractile out pouching from the basal left ventricle suggestive of a LV diverticulum. Coronary angiography showed normal coronaries with the LV angiogram showing the left ventricular out pouching with a narrow neck consistent with the findings of a diverticulum. Diverticulectomy with Dacron patch closure adopting the Dor procedure was done. Postoperative course was unremarkable and patient symptoms improved.