Niemann-Pick disease Type C (NPC) is a rare neurodegenerative disorder caused by recessive mutation in NPC1or NPC2 gene that exhibits lysosomal accumulation of unesterified cholesterol and glycolipids. Although several new pathological findings have been reported using patient-derived fibroblasts and animal models, the cure of NPC remains elusive. To understand more detailed pathological changes, we attempted to develop a neuropathological model using NPC patient-derived iPSCs.All iPSCs were maintained in StemFit AK02N. For neural induction, iPSCs were cultured in PSC Neural Induction Medium with Geltrex membrane matrix. For subsequent neuronal differentiation, these neural stem cells (NSCs) were reseeded onto iMatrix-511 coated wells in Neuronal Differentiation Medium. Characterization of these differentiated cells were assessed by PCR and immunofluorescence staining. iPSCs-derived NSCs showed high expression levels of neural stem cell markers, whereas there is no difference in differentiation status between controls and patients.However, abnormal accumulation of lipid-droplets and lysosomes were observed in NPC patient-derived NSCs. Subsequent maturation of NSCs showed neurite outgrowth and expression of neuronal marker. Although there is no difference in maturation status, neurite density were reduced in NPC patient-derived neurons. Furthermore, abnormal accumulation of lysosomes were observed in NPC patient-derived neurons.These results indicate that the neuropathology could be reproduced by this differentiation method.