Pantothenate kinase-associated neurodegeneration (PKAN) is a rare, autosomal recessive neurodegenerative disorder caused by mutations in the PANK2 gene located on chromosome 20p13. The mutation leads to iron deposition in the basal ganglia region of the brain, which causes progressive exacerbation of extrapyramidal symptoms, speech disorders, psychiatric symptoms, cognitive hypoplasia, and retinitis pigmentosa [1]. Here, we report a case of adult-onset atypical PKAN in a patient with psychiatric disorder as the first symptom, followed by dystonia, dysarthria, gait abnormalities, and limb tremor. Neuroimaging showed a typical “tiger's eye sign”. The patient was found to have a heterozygous PANK2NM_1386393.1:c.1172T>A(p.Ile391Asn)/ c.1039G>C(p.Asp347His) mutation by whole-exon sequencing. In addition, we review the pathogenesis, genetic features, clinical manifestations, imaging characteristics, and therapeutic strategies of PKAN patients.