A 22-year-old man was admitted with a rapidly enlarging soft mass on the left chest wall, which was diagnosed as lipoma by postoperative pathology. A chest roentgenogram revealed a defect of the fourth rib, scoliosis, dextrocardia, and diaphragmatic hernia. A computed tomographic scan showed maldevelopment of the pectoralis major and minor muscles. This is the first reported case of Poland syndrome with ipsilateral lipoma of the chest wall. Dextrocardia associated with Poland syndrome may be considered dextroposition, rather than a dextroinversion, and it may arise as a result of Poland syndrome.