This is a 15-year-old female who presented with sudden onset left flank pain associated with nausea and vomiting and a history of weight loss. Radiological investigation revealed a large non-obstructive tumor involving the lower pole of the left kidney which was primarily thought to be a renal cell carcinoma. She underwent left open radical nephrectomy with adrenalectomy. Histopathology of the resected specimen showed features of Ewing’s sarcoma of the kidney which was confirmed by cytogenetic analysis. This is a rare disease especially in the pediatric group and in reporting such a rare case we hope it helps in identifying a potential course of the disease and its response to the involved treatment.

A 44-year-old female presented to the urology clinic with flank pain and tenderness. After full assessment, the patient was booked for surgery for partial nephrectomy and the patient was diagnosed with renal cell carcinoma (RCC) chromophob type. Six months later, the patient came back for follow-up; a mass was detected on the same kidney. Radical nephrectomy was performed to excise what is thought to be a recurrence of RCC and the tissues were sent to pathology. The postoperative pathology report confirmed the presence of xanthogranulomatous pyelonephritis rather than RCC recurrence.

HighlightsRenal lymphangiomatosis is a rare congenital benign disease of renal lymphatic system.Here we are presenting a very rare form of disease, which is bilateral form.Disease can be found incidentally or with different signs and symptoms.Initial radiological work up will raise suspicion of renal tumor.Disease can be diagnosed and followed by imaging with an expert radiologist.Management usually analgesia unless a complex case or directed to the complications.