A case of a two-weeks-old female infant is reported who was born with an embryonal rhabdomyosarcoma in the size of more than a man's fist that was located in the area of the left half of the face. The tumor provoked an extreme disfiguration by its rapid postpartial growth when metastases had already developed. A palliative operation in order to maintain respiration and nutrition was performed when exitus letalis occured in hemorrhagical shock and consecutive circulatory break-down.