Renal cell carcinoma with rhabdoid features: A rare aggressive and fatal variant

Renal cell carcinoma with rhabdoid features is a rare histopathologic variant recently documented. It is a very aggressive tumor and associated with a higher mortality rate and poor prognosis. A 22 years old female patient presents with a rare case of clear cell renal cell carcinoma with rhabdoid features successfully managed with right radical nephrectomy and paracaval lymph node excision. The patient was alive, healthy and three years of flow up for the patient was free from metastasis. Despite rhabdoid features are lethal tumors and are associated with higher grades, radical nephrectomy with lymph node dissection increases survival rate.