We examined the Onufrowicz nucleus (Onuf's nucleus) of ten sporadic amyotrophic lateral sclerosis (ALS) patients with light and electron microscopic and immunohistochemical methods. Neurons in the Onuf's nucleus of ALS patients were better preserved than those in anterior horn cells. However, some showed morphological changes in the nucleus, namely, central chromatolytic changes, Bunina bodies, ubiquitin-positive filaments and spheroids. The Onuf's neurons of ALS patients showed more argentophilia than those of non-ALS patients. Electron microscopic observations revealed that neurofilaments were relatively more numerous in the Onuf's neurons of ALS patients. Bunina bodies and degenerated neurites were also seen in the Onuf's nucleus. In conclusion, the Onuf's nucleus in sporadic ALS patients showed some morphological changes similar to those noted in anterior horn cells.