Clear cell atypical fibroxanthoma (CCAFX) is a rare variant of atypical fibroxanthoma (AFX), a pleomorphic dermal tumour associated with a good prognosis. A 67-year-old man presented with a rapidly growing nodule on the ear, which had appeared over a 2-week period. Sections showed an ulcerated nodule composed of pleomorphic spindled and polygonal cells with clear cytoplasm, invested by a delicate vascular stroma, reminiscent of clear cell renal cell carcinoma. Numerous mitotic figures were seen. The tumour cells stained with vimentin, CD68 and CD99 and were cytokeratin-negative. The immunohistochemical and ultrastructural features supported a diagnosis of CCAFX. The diagnosis of CCAFX requires the exclusion of other pleomorphic clear cell tumours that can occur in the skin by using a combination of morphology, immunohistochemistry and electronmicroscopy. Murali R, Palfreeman S. Clear cell atypical fibroxanthoma - report of a case with review of the literature.