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Dilated cardiomyopathy (DCM) is a hereditary heart disease characterized by abnormal dilation of cardiac chambers, impaired cardiac function, and heart failure. Genetic testing has identified numerous genetic mutations associated with DCM, but its impact on patient care is still uncertain. Our aim is to describe the characteristics, management, and follow-up of patients with DCM who underwent genetic testing and to compare outcomes based on the presence of mutations. This is a local registry analysis of 53 patients with DCM who underwent genetic testing between March 2013 and October 2021. Genetic testing was performed in the same reference laboratory, and a panel of 80 major genes was analyzed. Clinical and paraclinical characteristics, management, and follow-up were collected. The study included a total of 53 patients, with a sex ratio of 1:1. The median age at diagnosis was 49 years, and the median follow-up duration was 40.5 months. The results showed that 43% of genetic tests were positive, with 11 identified genes, with TTN, LMNA , and MYH7 being the most frequently mutated genes. There was no significant difference between the mutated and non-mutated DCM groups in terms of clinical or paraclinical features. However, for a comparable LVEF, mutation carriers had more events during their follow-up than non-carriers (57 vs. 16%, P < 0.01). The genes LMNA, MYH7, DSP , and ABCC9 were the most associated with events. In addition, there was a significant correlation between mutation carriers and the occurrence of rhythm events (Spearman coefficient of 0.495) but no significant correlation between the presence of mutations and LVEF (Spearman coefficient of 0.125). In this study, it was not possible to distinguish patients with DCM with mutations from those without identified mutations. This finding reinforces the hypothesis of a multifactorial and non-Mendelian pathology. The risk stratification of sudden death is a complex task in DCM. Mutation carriers would have more events, particularly rhythm events. Future technological advances could also improve the identification of genetic risk factors and facilitate the implementation of personalized therapies for patients with DCM. [ABSTRACT FROM AUTHOR]