Rhabdomyosarcomas are malignant neoplasms with striated muscle differentiation. This is the most common type of soft-tissue sarcoma in children, but occurs rarely in adults. Its occurrence in liver is infrequent. We report a case of primary hepatic embryonal rhabdomyosarcoma in a 67-year-old man. The tumour was occupying the left lobe of the liver with large component of lesion seen bulging in left subhepatic space indenting over the stomach, compressing the pancreas and gall bladder. A percutaneous biopsy was performed which revealed embryonal rhabdomyosarcoma. He underwent a successful left lobectomy with complete resection of the tumour, followed by adjuvant chemotherapy. The patient is free of disease at 24 months of follow-up. Hence, an early diagnosis, prompt surgical resection with negative resection margin along with adjuvant chemotherapy can provide complete remission. This case is extremely rare both due to the age of the patient at presentation and location of the tumour.
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