OBJECTIVE  : Poland-Moebius syndrome is a rare disorder that consists of both Poland anomaly and Moebius syndrome features. The etiology is unclear, however it is thought to be secondary to vascular defect during sixth week of gestational age. The purpose of this report is to describe a case of Poland-Moebius syndrome and it is emphasized on the possible serious condition.  
CASE PRESENTATION : A four months old male baby came to pediatric-endocrinology clinic due to multiple congenital anomalies. Physical examination revealed right major pectoral muscle atrophy with nipple hipoplasia, and symbrachydactyly of the right hand that were associated with Poland anomaly. There were also findings of bilateral facial paralysis with micrognathia, esotropia, and brachicephaly which were correlated with Moebius syndrome. Thus, diagnosis of Poland-Mobius syndrome was suggested. There was no history of teratogenic exposed during pregnancy. Chromosomal analysis showed a normal 46,XY karyotype. At the age of eight months old, he developed recurrent pneumonia in association with further endoscopy findings of laryngomalacia and narrow airway. These conditions caused severe respiratory distress and was fatal to this patient.
CONCLUSION  : We report clinical manifestations of Poland-Moebius syndrome that correspond to airway complication and became fatal to this case.