Nikolaus Friedreich (1825–1882) presented clinical findings in six patients with a severe hereditary disorder of the nervous system and secured full autopsies in four of them. He was fascinated by the spinal cord lesions in the siblings of two unrelated families, and in the first three of his five long articles stressed the destruction of the dorsal columns. He recognized the relatively minor symmetrical lesions of the anterolateral fasciculi but did not separate dorsal spinocerebellar tracts (Flechsig's bundles) and corticospinal tracts. Although he studied the dorsal spinal roots in great detail and established their principal abnormality, namely, axonal thinning without axonal loss, he reported dorsal root ganglia as entirely normal. He made an insightful description of atrophic neurons in the gracile nuclei (clavae) but overlooked the invariable atrophy of the dentate nuclei. He followed the families over a period of 14 years, but acknowledged the hereditary nature of the disease only very late. He proposed a developmental defect for the medulla oblongata, retaining his interpretation that the spinal lesion was inflammatory. This review honors Friedreich for his insight into a ‘new’ disease in the late 19th century and updates his neuropathological findings. It is remarkable that Friedreich also described the abnormal hearts in the disease that now bears his name since hypertrophic cardiomyopathy is now recognized as the main cause of death in Friedreich's ataxia.