<p>Cardiomyopathy is a condition where cardiomyocytes are defective, leading to failures in cardiac function. Cardiomyopathy has been classified into three main groups, with several subtypes based on molecular and/or systemic alterations. The presentation of the disease is age- and sex-independent, with an incidence ranging from 1/250 to 1/5000, depending on the subtype. Critically, cardiomyopathy may result in sudden death, and phenotypes can overlap with other pathophysiology. Thus, molecular methods are fundamental for differential and early diagnosis. Although several genetic profiling studies have been conducted in diverse populations, efforts are still needed to identify new associated genes and variants and confirm previously reported variants. Therefore, this study aimed to report the mutation profiles of 40 Turkish patients with different subtypes of cardiomyopathy using clinical exome sequencing (CES). Our results showed that males were more affected than females. Additionally, patient ages ranged drastically from 5 to 74 years. Among the subtypes, hypertrophic cardiomyopathy was the most prevalent. Finally, myosin-binding protein C3 (<i>MYBPC3</i>) was the gene most frequently varied in the Turkish cohort, with 24 variants of unknown significance potentially linked to cardiomyopathies. Further molecular studies and cohort reports are needed to confirm the pathogenicity of these variants.</p>