Poland syndrome is characterized by unilateral aplasia or hypoplasia of the sternocostal portion of the pectoralis major muscle and ipsilateral syndactyly. In some cases other associated anomalies, including renal malformations, dextrocardia, and vertebral abnormalities, have been reported. We report a 7-month-old girl with Poland syndrome who also presented with ipsilateral renal agenesis. This report suggests that renal structural anomaly may be an integral part of this syndrome. We recommend renal imaging studies be performed on all children with Poland syndrome.