Background:

Undifferentiated embryonal sarcoma of the liver accounts for 9-13% of malignant tumors in the pediatric age group, and is the third primary malignant neoplasm of the liver in children. However, few cases are reported in the literature. It may manifest with fever, weight loss, pain, and abdominal tumor sensation. In addition to pathology and immunohistochemistry, imaging studies are the appropriate tools for diagnosis.

Case report:

We present the case of a 6-year-old female patient diagnosed with undifferentiated embryonal sarcoma of the liver by surgery and subsequent biopsy results.

Conclusions:

When reviewing the literature, we found that this type of malignant neoplasm is not frequent in children. However, it is important to consider this type of tumor as a cause in those cases of hepatomegaly in the pediatric age.