Poland's syndrome is characterized by hypoplasia or absence of the breast of nipple, hypoplasia of subcutaneous tissue, absence of the costosternal portion of the pectoralis major muscle, absence of the pectoralis minor muscle, and absence of costal cartilage or ribs. The syndrome involves a spectrum of conditions ranging from simple absence of pectoralis major muscle and breast to the defects noted by Poland as well as brachysyndactyly, ectromelia. Furthermore, there have been reports of Poland's syndrome associated with malignancies such as leukemia, malignant lymphoma,(1) and leiomyosarcoma.(2) We have experienced a 22-year-old woman presenting with asymmetric breast and abnormal position of nipple and accessory breast suggested Poland's syndrome. Treatment consisted of tissue expansion of hypoplastic breast and free nipple graft and excision of accessory breast. The purpose of the study was to review the present knowledge of Poland's syndrome and literature.