Primary primitive neuroectodermal tumor (PNET) of the liver is a rare disease with aggressive behavior and poor prognosis. We report a case of a PNET of the liver in a 13-year-old boy. The patient was admitted to the hospital with fever and abdominal pain. Abdominal CT and MRI revealed a 5.5 cm sized, septated, non-enhancing mass in the hepatic hilum. The patient was initially diagnosed with an inflammatory pseudotumor. Despite 9 days of antibiotic therapy, the patient's clinical symptoms did not improve. A liver biopsy was performed in the interest of formulating a differential diagnosis. This procedure revealed tumor cells positive for CD99 on immunohistochemistry. The patient was diagnosed with a PNET.