Creutzfeldt-Jakob disease is the most common disorder among the very rare human transmissible subacute spongiform encephalopathies. Sporadic, familial or iatrogenic forms of the disease can be seen. The common presentations of the disease include rapidly progressive cognitive decline, behavioral changes, visual disturbances and cerebellar dysfunction. Hyperparathyroidism and toxicity of lithium and bismuth have been reported to induce similar symptoms and EEG findings, leading to a Creutzfeldt-Jakob like syndrome. We report a very rare case of Creutzfeldt-Jakob disease presenting with hyperparathyroidism and generalized tonic status epilepticus.