Lipoid proteinosis is an infrequent disease characterized by the deposition of a PAS-positive diastase-resistant hyaline material in the skin and respiratory tract, although it can also be deposited in internal organs, in a generally asymptomatic manner. The earliest clinical manifestation is hoarseness. Clinical cutaneous manifestations come later, in the form of hyperkeratotic lesions located on the trunk, elbows, axillae, groins, backs of hands, palms and soles. A lesion typical of the disease is moniliform blepharosis, which consists of beaded papules along the eyelid margins. Also characteristic is the presence of comma-shaped intracranial calcifications in the temporal lobes. The course of the disease is progressive, with a normal life expectancy. It affects men and women equally, with worldwide distribution. The diagnosis is based on the clinical symptoms and the histology. At this time, there is no effective treatment for the disease. We present a case of lipoid proteinosis in a 23-year-old woman, with typical clinical and histological characteristics.