Congenital ventricular aneurysm is a very rare cardiac anomaly. A diagnosis can be made during the prenatal period using fetal echocardiography. This study presents a very rare apically located left ventricular aneurysm case, and the relevant literature was reviewed and discussed. In this case, a 35-year-old, gravida 2, parity 1 preg-nant woman at 24 weeks of gestation, displayed a wide aneurysmal image in the left ventricular apical wall on fetal echocardiography. There was a 1.79 mm muscular ventricular septal defect at the apical region of the interven-tricular septum. In the course of the color Doppler ultrasonography examination, an aberrant fibrous band within the left ventricle and consequent turbulent flow during systole were observed. The baby, born via cesarean section at 37 weeks of gestation, is now in its postnatal seventh month. However, during echocardiographic follow-ups, changes have been observed, including mild to moderate mitral insufficiency and a decrease in systolic function. Despite these findings, the clinical condition remains asymptomatic. It is of great importance to use a multidis-ciplinary approach in managing these rare cases that could lead to potential adverse outcomes during the antena-tal or postnatal periods.