Lipoid proteinosis is a rare hereditary multisystemic familial disorder characterised by intracellular deposition of hyaline material (Rao and Koppada in Indian J Dermatol Venereol Leprol, 2015
Shah and Shah in J Oral Maxillofac Pathol 26:236-241, 2022). The hallmark findings are pox like scarred skin lesion, hoarseness of voice and selective brain parenchymal calcification (Rao and Koppada in Indian J Dermatol Venereol Leprol, 2015). We present a case of biopsy proven lipoid proteinosis with typical radiological findings.