Adult polyglucosan body disease is a recently established clinicopathological entity, with few cases reported in the literature. In this paper we describe a case of a 46-year-old man who had died to a pancreatic cancer whose neuropathological study revealed a massive accumulation of polyglucosan bodies in the cerebral white matter, brainstem, cerebellum and spinal cord consistent with the adult polyglucosan body disease. We also discuss the pathological criteria used to differentiate this disease from others in which polyglucosan bodies may accumulate in the nervous system.