Basal cell carcinoma (BCC), classified as a non-melanoma skin cancer (NMSC), is the most common type of skin cancer. Histopathologically, BCC can be classified into indolent-growth subtypes (nodular and superficial) and aggressive-growth subtypes (morphaeform, infiltrative, micronodular and basosquamous). Additionally, uncommon variants have been described in the literature including adamantinoid, granular, clear cell, and BCC with matrical differentiation (BCCMD).

To the best of our knowledge, there have only been 32 cases, including the present one, of BCCMD reported in the literature. BCCMD shows differentiation towards hair matrix and is characterized by islands of basaloid cells along with shadow cells, which are characteristic markers of pilomatricomas and also found in other entities.

We report a case of BCCMD that presented as a nasal skin lesion that had been persistent for one year. The lesion had been resected several times over an 8 year period at other institutions and had been reported as a BCC. The patient underwent completion rhinectomy at our institution, and final pathology result showed BCC with matrical differentiation. PET scan was performed post-op which showed a left neck active submandibular mass. Biopsy confirmed malignancy. A left neck dissection was performed, and final pathology results confirmed metastatic BCCMD.

In conclusion, while BCCMD might be considered to show similar behavior as other BCCs, which are often only locally invasive, our study demonstrates its potential for metastasis. Therefore, it is of critical importance to differentiate it from the other subtypes.