Basal cell carcinoma (BCC) may exhibit diverse differentiation. In BCC with matrical differentiation, islands of shadow cells, which are characteristic of a pilomatricoma, are located within the tumor. This is an extremely rare variant and to our knowledge, only two review articles and one case report have been published. We recently experienced a case of BCC with matrical differentiation in a 45-year-old Caucasian male. This tumor should be differentially diagnosed from other tumors, particularly a pilomatricoma and pilomatrix carcinoma.