Progressive cavitating leukoencephalopathy has been described in patients with pathogenic variants in NDUFV1, as well as the nuclear genes NDUFS1, NDUFV2, and NDUFA1, which are also involved in the formation of complex I subunits [1, 2, 5, 8, 11, 13, 14]. Here, NDUFV1 is linked to Leukoencephalopathy.