Background <p>Andersen-Tawil Syndrome (ATS) is an autosomal dominant disorder defined by a classic triad of dysmorphic features, episodic muscular weakness, and ventricular arrhythmias. Notably, gender appears to significantly influence clinical outcomes, with females constituting the predominant affected demographic and exhibiting poorer cardiovascular prognoses.</p> Methods <p>This study seeks to outline the gender-specific clinical features of ATS, with an emphasis exclusively on patients exhibiting cardiac manifestations. A total of 114 case series and reports were collected from 1994 to 2024 from Scopus, Embase, PubMed, Web of Science, CINAHL, and Cochrane Library. Only patients exhibiting cardiovascular manifestations were included in the study, excluding cohorts without single patient characteristic descriptions. Analysis was performed using the available data for each variable.</p> Results <p>The studied population comprised 259 cases, of whom 176 were female. The analysis demonstrated that females are less likely to present with the characteristic triad than males (<i>p</i> = 0.03). Additionally, female patients were less likely to present with periodic paralysis than males (<i>p</i> = 0.02). Overall, premature ventricular contractions (PVCs) were the most common arrhythmia, bigeminy being the most common presentation. Polymorphic PVCs were more prevalent in females than males (<i>p</i> = 0.01). Females experience more ventricular tachycardia episodes than males, especially bidirectional (<i>p</i> = 0.01). Thirty-seven patients had an episode of cardiac arrest, either fatal or non-fatal. Females were more prone to develop sudden cardiac arrest compared to males (<i>p</i> = 0.02).</p> Conclusion <p>ATS females with cardiac manifestations may require closer follow-up due to the higher risk of developing life-threatening arrhythmias and cardiac arrest than males.</p> Graphical Abstract <p></p>