Non-compaction of the ventricular myocardium (NCVM) is an under-diagnosed cardiomyopathy. Patients diagnosed with NCVM at the King Abdulaziz Cardiac Centre, Riyadh, KSA from January 2000 to July 2004 and at the Sudan Heart Centre from August 2004 to July 2007 were included. Fifty-two patients with NCVM were identified (22 per 10 000 echocardiograms). Patients were divided into three groups, namely, group 1: isolated NCVM (21 patients), group 2: NCVM associated with congenital heart disease (CHD) (26 patients), and group 3: NCVM associated with mitral regurgitation (MR) (seven patients). Group 1 included 14 females and four males. Five patients (27%) had a positive family history with a lethal outcome in five other siblings; 14 patients (76%) presented with myocardial dysfunction and two had left ventricle thrombus. Group 2 included CHD; the most common pathologies were ventricular septal defects (VSD), pulmonary and tricuspid atresia and hypoplastic left heart syndrome. Sixteen patients (61%) had myocardial dysfunction, seven had surgical repair/palliation, and four (80%) developed serious post-operative complications. Group 3 included seven patients with MR associated with deformity of the anterior mitral leaflet and malcoaptation. Myocardial function was preserved in all patients with this pathology. In four patients of the whole cohort there was clinical as well as echocardiographic improvement. In two patients, left ventricular hypertrophy was noted. There were significantly more females in the group with isolated NCVM than in the group with associated CHD (p = 0.03, odds ratio = 4.2, 95% CI = 0.529-16.1). We presented the largest series of NCVM in our area and found it to be not as rare as was thought, with females being more affected. Spontaneous improvement and left ventricular hypertrophy were unique features, and mitral valve deformity leading to MR was an established association.