Chronic inflammatory demyelinative polyneuropathy (CIDP) is an acquired neuropathy, presumably of immunological origin. Its clinical presentation and course are extremely variable. CIDP is one of the few peripheral neuropathies amenable to treatment. Typical cases associate progressive or relapsing-remitting motor and sensory deficit with increased CSF protein content and electrophysiological features of demyelination. In other instances the neuropathy is predominantly or exclusively motor or sensory, CSF normal and electrophysiological studies fail to show evidence of demyelination. In such cases conventional diagnostic criteria are not filled yet the patient may respond to immunomodulatory treatments. In this paper we review the diagnostic pitfalls and clinical variants of CIDP to illustrate the problems that may arise. The different therapeutic options are reviewed. Axon loss associated with demyelination is the most important factor of disability and resistance to treatment.