PLA2G6-associated neurodegeneration (PLAN) is a rare autosomal recessive disorder with broad clinical spectrum, encompassing extrapyramidal, cognitive, and behavioural features, even among patients carrying the similar variant. We describe three young males, all harbouring same homozygous c.2222G > A (p.Arg741Gln) variant in PLA2G6, each exhibiting distinct phenotypes: one developed early-onset parkinsonism, another with dystonia-parkinsonism accompanied by behavioural changes, and the third had psychiatric symptoms progressing to parkinsonism with cognitive decline. The phenotypic heterogeneity may arise from modifiers beyond mutation location, including variable expressivity, epigenetics, and oligogenic effects as possible contributors. These insights may hold therapeutic relevance, particularly in guiding developing future disease-modifying therapies.