Introduction. Clear cell basal cell carcinoma (BCC) is an uncommon and unusual variant of BCC, which is characterized by a variable component of clear cells. The pathogenesis of this histological variant and its clinical significance has not been clarified. Differentiation of this uncommon variant of BCC from other clear cell tumors is important for the treatment. Case Presentation. A 65-year-old male presented with a 0.9 cm dome-shaped lesion on his upper chest. A shave biopsy revealed a dermal basaloid tumor that comprised nests with a peripheral palisading pattern, retraction artifacts, and striking clear cell changes. Histopathologic examination, along with findings from immunohistochemical studies and special staining of the clear cells, supports the diagnosis of clear cell basal cell carcinoma. Conclusion. Clear cell BCC is a rare and unusual variant of BCC. The underlying pathogenesis of this subtype is unclear; however, accurate identification may affect treatment and prognosis.