Leukoencephalopathy with brain calcifications and cysts (LCC), first described by Labrune et al. in 1996 [1], is a rare autosomal recessive genetic disorder caused by biallelic mutations in Small Nucleolar RNA, C/D Box 118 (SNORD118), a non-protein-coding small nucleolar RNA gene on chromosome 17p13.1 [2]. The gene discussed is SNORD118; the disease is Leukoencephalopathy.