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Kufs disease (KD), adult neuronal ceroid lipofuscinosis (NCL), differs from its common childhood forms by its late onset and preserved vision and is subclassified based on phenotype as type A, manifesting as progressive myoclonus epilepsy (PME), and type B, manifesting as dementia with motor involvement. cWe report a patient with type B KD with dementia and parkinsonism who showed a remarkable life-changing response to levodopa, the first documented response of its kind in this phenotype caused by CLN6 mutation. c Cerebellar atrophy on MRI brain, photoparoxysmal response on EEG to low-frequency intermittent photic stimulation, and giant somatosensory evoked potentials are useful diagnostic clues for KD, especially given the lack of specificity of peripheral tissue biopsy findings in the adult population. c KD should be considered in a patient with slowly progressive dementia presenting in early adulthood with the above features and genetic analysis is suggested for confirmation. Oy-sters cCurrent well-established genes for type A KD are CLN6 and DNAJC5 and for type B cathepsin F (CTSF) mutation. c Peripheral tissue biopsy in KD is not reliable and overreliance on electron microscopic inclusion deposits may overdiagnose KD in adults, unlike in children.