Clear cell sarcoma of the kidney is the most frequently misdiagnosed renal tumor in children. The majority of tumors present the classic histologic pattern, which allows a definitive diagnosis. However, there are unusual cases with lack of “clear” appearance of tumor cells, predominance or exclusive presence of variant histologic patterns, and presence of “neoplastic” appearing entrapped tubules. Furthermore, a small biopsy specimen may not show the classic histologic pattern. These tumors present a diagnostic challenge for the practicing pathologist who should be aware of the deviations from the classic histologic features in order to make a correct diagnosis.