A 2(1/2) year old girl was presented with an acute, painless orbital swelling without signs of local or systemic inflammation. CT and MRT scanning revealed a tumor which by open biopsy disclosed embryonal rhabdomyosarcoma. By means of this case we present the current diagnostic and therapeutic approaches for childhood soft tissue sarcomas. At present our patient receives a poly-chemotherapy which has already shown a good response. For local tumor control, a complete surgical resection as well as radiotherapy may be indicated and have to be considered carefully with respect to the possible late effects. This case demonstrates that in a child with a unilateral, progressive orbital tumor, the differential diagnosis of a soft tissue sarcoma has to be ruled out.