A case of lipoid proteinosis was subjected to clinical, histologic and electron microscopic study. Palpebral biopsies showed a large dermal infiltration composed of closely interwoven granulofilamentous material in the superficial and deep dermis. Collagen fibers were normal. Fibroblasts seemed to play an active role in the synthesis of this accumulating substance. Around the dermal capillaries there was a prominent concentric multilaminalion of the basal lamina which was considered a reaction of the endothelial cells to the irritation caused by compression of the accumulating substance.