PLA2G6 (phospholipase A2 group VI) encodes a calcium-independent phospholipase involved in lipid metabolism and mitochondrial homeostasis 1. The protein mediates detoxification of oxidized phospholipids by cleaving acyl tails from the glycerol backbone, converting oxidized phosphatidylethanolamine to lyso-phosphatidylethanolamine and regulating ferroptosis and mitochondrial function 23. PLA2G6 is activated by the mitochondrial protease LACTB, which cleaves and activates the phospholipase to control lipid-mediated kidney and metabolic dysfunction 2. Pathogenic PLA2G6 variants cause PLA2G6-associated neurodegeneration (PLAN), a clinically heterogeneous spectrum of autosomal recessive disorders 1. These include infantile neuroaxonal dystrophy/neurodegeneration with brain iron accumulation (NBIA) 2A, atypical neuronal dystrophy/NBIA2B, and adult-onset dystonia-parkinsonism (PARK14) 41. PLA2G6 mutations are a recognized cause of early-onset Parkinson's disease, particularly in autosomal recessive inheritance patterns 567. The pathophysiology involves mitochondrial dysfunction, α-synuclein aggregation, and impaired membrane homeostasis 1. Genetic testing is clinically important for differential diagnosis in early-onset and familial parkinsonian syndromes 8.