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Neurofibromatosis type 1 (NF1) is a benign peripheral nerve sheath tumor, which can primarily cause neurocutaneous manifestations. We herein present a rare case of periportal neurofibromatosis with intrahepatic, retroperitoneal and pelvic involvement. The patient underwent abdominal ultrasound (US), magnetic resonance imaging, and a positron emission tomography-computed tomography. Despite these various imaging features, a diagnosis of lymphomas could not be entirely excluded. The patient therefore underwent US-guided biopsy, and a diagnosis of intrahepatic neurofibromatosis was made. Through retrospective analysis of this case along with literature review, we concluded that distributing along the Glisson’s sheath may be a characteristic in the diagnosis of NF that involves liver.