The ELST shown in Figure 1 occurred in the left temporal bone of an 8-year-old boy who presented with profound sensorineural hearing loss, intermittent vertigo, and vHL based on mutation analysis that showed a G-to-C nucleotide 601 mutation in the vHL gene on chromosome 3. High-resolution computed tomographic imaging using a bone-window protocol demonstrates a destructive mass involving the posterior aspect of the left petrous temporal bone centered midway between the internal auditory canal and the sigmoid sinus. Bony erosion extends into the posterior semicircular canal, and there are areas of stippling and central calcification within the mass suggestive of an aggressive, erosive process. Magnetic resonance images of ELSTs may demonstrate enhancement on precontrast T1-weighted images and heterogeneous enhancement on T2-weighted images. Larger lesions may show flow voids. Although the diagnosis in this case was relatively straightforward on the basis of the combination of genetic screening studies and characteristic imaging features on computed tomographic scanning, a differential diagnosis of similar lesions should include meningioma, choroid plexus papilloma, paraganglioma, chondrosarcoma, and metastasis.