Left ventricular noncompaction cardiomyopathy (LVNC) is a rare form of familial cardiomyopathy attributed to failure of ventricular compaction during embryogenesis. Scant data exists regarding the management approach to pregnant patients with this condition. Additionally, few cases are reported in which both the mother and fetus are simultaneously affected by this condition during the antepartum period. This case is unique in that the patient carried a known genetic and echocardiographic diagnosis of LVNC, prior children born with confirmed LVNC by genetic and echocardiographic findings, and the current pregnancy affected by a perinatal diagnosis of LVNC via sonographic findings.