RESUMEN
Rhabdomyosarcoma [RMS] occurs infrequently in the liver. Rhabdomyosarcomas are malignant tumours that display features of striated muscle differentiation. They are the most common soft-tissue sarcomas among children. In adults however, these are very rare. We report a case of a primary embryonal rhabdomyosarcoma of the liver in a 17 years old boy. This was confirmed by histological examination using immunohistochemical analysis [LCA negative, desmin positive, myogenin focally positive and cytokeratin negative] and site was confirmed by PET CT scan. He received multiple chemotherapies including [doxorubicin, ifosfamide, dacarbazine; gemcitabine, paclitaxel; vincristine, actinomycin D, cyclophosphamide] but longest sustained stable disease was seen with gemcitabine-paclitaxel regimen. The patient died 31 months after the first presentation, secondary to complicated abundant abdominal progressive disease. The poor prognosis and early death of most previously reported cases imply the need for investigation of a more effective treatment method of this uncommon tumour