Poland syndrome is characterized by unilateral absence or hypoplasia of the pectoralis muscle and variable degree of ipsilateral hand anomalies. Möbius syndrome is a congenital neurological disorder characterized by complete or partial facial paralysis. Although the pathogeneses of these diseases are not well-characterized, diminished blood flow to the affected side is thought to play a role. A male infant weighing 2.670 g was born at 38+3 weeks of gestation with left facial paralysis, left chest wall defect with dextrocardia, and symbrachydactyly between the second and third fingers. The combination of Poland-Möbius syndrome is rare, and only 2 cases associated with dextrocardia have been reported worldwide. Here, we report the first case of Poland-Möbius syndrome associated with dextrocardia in Korea.