Objective: To report a case of congenital right pulmonary hypoplasia presenting as cardiac dextroposition on prenatal ultrasonography. Case Report: A 29-year-old woman with fetal dextroposition visited this institution at 24 weeks of gestation. Prenatal fetal echocardiography and detailed ultrasonography could not detect fetal anomaly except for fetal dextroposition. However, congenital pulmonary hypoplasia was diagnosed postnatally. Conclusion: Primary unilateral pulmonary hypoplasia should be suspected in cases with abnormal heart position of an unknown origin and fetal magnetic resonance imaging (MRI) could be an additional option for prenatal diagnosis of primary pulmonary hypoplasia.