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Provided is an overview of the pathology, clinically defining features, and subcategorization of peripheral nerve amyloidosis. This condition is pathologically hallmarked by infiltrative amyloid deposition in varied peripheral neural tissue localizations with associated clinical manifestations. Peripheral nerve involvement is a frequent presenting feature of systemic amyloidosis (Kyle et al., Peripheral Neuropathies, Saunders, Philadelphia, PA, 2,427–2,451, 2005). As with other organ systems, the diagnosis of amyloid involvement of peripheral nerve is often delayed because the clinical features may mimic many varieties of peripheral neuropathy. Therefore, pathologic discovery of unsuspected amyloidosis is common. The particular amyloid protein can determine the clinical course and preferred treatment modality, as well as potentially alert patients to familial predisposition. Advances in laboratory technology, including most notably mass spectrophotometric evaluation of nerve, continue to improve the accuracy and sensitivity of amyloid subtyping (Adams, J Neurol 248(8), 2001; Benson and Kincaid, Muscle Nerve 36(4), 2007; Klein et al. Arch Neurol 68(2), 2011). Appropriate directed peripheral neural biopsy can therefore be instrumental in identifying the diagnosis providing the specific amyloid type, informing prognosis, and directing care in each case.