Poland syndrome is a rare malformationcharacterized by agenesis of the pectoralis major muscle and breast agenesis associated or not with anomalies of the homolateral upper limb. We described a unique case of complete Poland syndrome with hepatic evisceration and diaphragmatic eventration in a female newborn, confirmed by a thoracic computed tomography scan. The patient underwent a thoracotomy with the repair of the chest wall. The follow-up was uneventful. Poland syndrome is a rare congenital malformation with varying degrees of thoracic and homolateral upper limb malformations. It is the first case of complete Poland syndrome reported in the literature.