A variant case of Poland syndrome is reported, with the patient presenting major unilateral anomalies in the sternocostal head of the left pectoralis major muscle and a shorter left arm and hand. No pre-natal history of attempted abortion or using toxic or teratogenic drugs could be elicited. The variability of the upper extremity anomalies and the frequency of major ipsilateral defects of the skeletal and genite-urinary systems, the associated diseases, and the diagnosis and management of these patients are briefly discussed.