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Double chambered left ventricle (DCLV) is an uncommon congenital heart condition typically identified incidentally, with the majority of patients showing no symptoms and experiencing a benign course. It is crucial to differentiate DCLV from other abnormalities like diverticulum or aneurysm, which can have significant clinical implications. Due to the limited available data, our understanding of the natural progression, prognosis, complications, and treatment options for this rare condition is poorly defined. A review of the medical literature reveals the use of various overlapping terms when describing DCLV. In our case report, we present the evaluation of a young male who sought medical attention for palpitations. Initially, DCLV was diagnosed through 2D echocardiography. However, subsequent cardiac magnetic resonance imaging (CMR) did not confirm the presence of two distinct chambers but instead revealed an anomalous apical basal muscle bundle (ABMB) and atypical left ventricular (LV) trabecularization that resembled DCLV., (© 2024 Wiley Periodicals LLC.)