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Congenital diverticulum of the ventricle is a rare cardiac malformation that causes cardiac abnormalities. The course of this disorder is variable, and the condition is potentially fatal in some cases. The most effective diagnostic methods for the left ventricular apical diverticulum are various imaging techniques. We present a patient with congenital left ventricular apical diverticulum who presented with dome-shaped ST-segment elevation in lateral leads. In our case, the dome-shaped ST-segment elevation in lateral leads contributes to the early diagnosis of the left ventricular apical congenital diverticulum.