Non-compaction of the ventricle (NCV) with a higher tendency to left ventricular involvement (NCLV) is a genetic disorder which can cause arrhythmias and cardiac arrest or remain asymptomatic. It is generally considered an isolated disease most frequently, while a few case reports have reported its association with cardiac anomalies. As the treatment strategies differ for NCV and cardiac anomalies, missed diagnosis of the concomitant cardiac diseases can result in poor response to treatment and prognosis. Here, we present 12 adult patients diagnosed with NCV and associated cardiovascular anomalies. By increasing the clinical suspicion and physician’s awareness about the possibility of the presence of other cardiovascular diseases with NCLV and using close examination and follow-up of the patients, we could diagnose this number of patients during 14 months of investigation. This case series emphasizes the need for increased awareness and attention of echocardiographers on the diagnosis of other cardiovascular diseases associated with NCV for a better response to treatment and improved patient prognosis.