Rhabdomyosarcoma (RMS) is a common, highly malignant, uniformly fatal childhood malignancy, which presents extremely rarely in the neonatal period; there are only a few reports about this tumor in this age group. While the primary tumor may arise virtually anywhere in the body, the extremity, orbit and genitourinary region are the most frequent sites; the retromammary region is extremely rare. Herein, we report a neonate with embryonal RMS arising from the anterior chest wall musculature at birth.