Immunohistochemical staining of hemangioblastoma was typically positive for vimentin, carbonic anhydrase, S100 protein, neuron-specific enolase, CD57, and inhibin, and negative for glial fibrillary acidic protein, cytokeratin, epithelial membrane antigen, CD34, HMB-45, desmin, and actin.18) The combination of negative cytokeratin and positive carbonic anhydrase IX is highly sensitive (90%) and specific (100%) for the diagnosis of hemangioblastoma.19) These features were present in this case. The gene discussed is DES; the disease is hemangioblastoma.