Isolated left ventricular noncompaction (ILVNC) is a cardiomyopathy caused by intrauterine failure of the myocardium to compact. Common clinical complications are heart failure, arrhythmias, and cardioembolism. A paucity of data exists relating to clinical and echocardiographic features of ILVNC in Africans. This study is a single-center, prospective case-control study, whereby subjects attending a dedicated cardiomyopathy clinic were screened for and diagnosed with ILVNC, provided they had no other associated structural heart disease and fulfilled all the accompanying echocardiographic criteria: (1) end-systolic ratio of noncompacted layer to compacted layer >2, (2) presence of >3 prominent apical trabeculations, and (3) deep intertrabecular recesses that fill with blood from the ventricular cavity visualized using color Doppler ultrasound. Fifty-four subjects were identified, age 45.4±13.1 years (mean±SD), 95% confidence interval 3.6 to 10.2, 55.6% male, and 63.0% New York Health Association Class II, and prevalence of LVNC in our clinic was 6.9%, 95% confidence interval 3.6 to 10.2. Heart failure because of systolic dysfunction was the most common clinical presentation (53 subjects, 98.1%). Left ventricular end-diastolic diameter was 61.4±7.2 mm (mean±SD) and ejection fraction 26.7±11.9% (mean±SD). Common sites of noncompaction were the apical (100%), midinferior (74.1%), and midlateral (64.8%) walls. Right ventricular noncompaction occurred in 12 subjects (22.2%). Pulmonary hypertension was documented in 45 cases (83.3%). Right ventricular dilation was noted in 40 subjects (74.1%), while right ventricular function was depressed in 32 (59.3%). Tricuspid S' was 9.6±2.8 cm/s (mean±SD). No echocardiographic features suggestive of ILVNC were noted in a healthy control group of African descent. ILVNC in patients of African descent can be characterized by biventricular abnormality and pulmonary hypertension, in addition to isolated left-sided abnormality.