Abstract
Neuronal intranuclear inclusion disease (NIID) is a relatively new entity identified as a progressive neurodegenerative disease characterised by eosinophilic hyaline intranuclear inclusions widely observed in neuronal and somatic cells. Renal biopsy from one of our patients with NIID showed lupus nephritis-like pathology. He was treated with steroids and angiotensin-converting enzyme inhibitors and his proteinuria improved. The present case highlights that immune-mediated glomerulonephritis can be a presenting feature of NIID, which can be controlled with proper treatment.