HighlightsRenal cell carcinoma (RCC) with t(6:11) (p21;q12) are extremely rare, fewer than 30 cases have been reported in literature.This study reports a case of 11 year old boy diagnosed with this rare tumor, with the aim of highlighting distinctiveimmuno-histologic features of this tumor otherwise diagnosed by fluorescence in situ hybridization technique.Histologically tumor has a distinct biphasic pattern, with “pseudorosette” formation.Tumor cells show immunopositivity for melanocytic markers, HMB45 and Melan A and are negative for epithelial markers CK and EMA.Knowledge of unique immunohistology is helpful in not misdiagnosing this rare tumor for a non epithelial tumor like angiomyolipoma.