We present the diffusion-weighted imaging (DWI) findings for a malignant peripheral nerve sheath tumor arising in a retroperitoneal plexiform neurofibroma in a patient with neurofibromatosis type 1. Signal intensity of the malignant area was high on DWI and low on the apparent diffusion coefficient map and differed from findings for the benign area. DWI enabled clear differentiation between malignant and benign areas of the tumor.