NBIA syndromes are caused by mutations in a number of different genes [1], such as PANK2, responsible for PKAN, or C19orf12 underlying mitochondrial membrane protein-associated neurodegeneration (MPAN) [8,9] In general, acanthocytosis has not been described in NBIA apart from PKAN where roughly 10% of PKAN patients are considered affected. This evidence concerns the gene PANK2 and neurodegeneration with brain iron accumulation.