Tumors of the adrenal medulla, extra-adrenal paraganglia of the sympathetic neuroendocrine system, and paraganglia of the head and neck are derivedfrom neural crest cells. These tumors manifest as growing masses and can cause endocrine dysfunction due to unregulated secretion of hormones. Tumors arising in the adrenal medulla (pheochromocytomas) and extraadrenal paraganglia (paragangliomas) usually present in adulthood. Their diagnosis is based on histologic features, immunohistochemical stains, and electron microscopy. These features are reviewed, as are familial syndromes and their associated gene mutations.