A 63-year-old man presented with a signet ring cell basal cell carcinoma of the right infraorbital area. This is the third reported case of this rare variant of basal cell carcinoma characterized by tumor cells containing large, hyalinized, eccentric, intracytoplasmic inclusions that compress nuclei into crescent or ring-shaped forms. Antibodies to both high and low molecular weight cytokeratins were strongly positive, staining the inclusions in a uniform fashion. Vimentin and actin antibodies did not stain the inclusions. These results support previous electron microscopic studies that show the inclusions to be aggregates of intermediate filaments blending into tonofilaments at their periphery. Although speculative, the formation of signet ring cells does not appear to be a degenerative or necrotic phenomenon, but probably a peculiar aberrant form of individual cell keratinization.