The syndrome of Poland is a rare congenital malformation, grouping, to varying degrees, musculoskeletal abnormalities of the chest wall and abnormalities of the ipsilateral upper limb. We report two observations collected of this rare syndrome whose etiology remains uncertain. It seems that a defect of vascularization during the fetal development is at the origin of the described anomalies. CT is the exam of choice, allowing a complete assessment of the lesion, to look for the associated malformations and to guide the therapeutic attitude which varies according to the sex and the severity of the attack.