Primary neuroendocrine tumours (NETs) of kidneys are extremely rare tumours. We report a case of a young female patient, who presented with complaints of hematuria and was diagnosed as a case of the right renal space-occupying lesion (SOL) on radiological imaging. She was advised for surgery, however, was lost to follow up. She remained asymptomatic and presented two years later again, pain abdomen, hematuria and abdominal mass. Imaging revealed a large space-occupying lesion in the right kidney pelvicalyceal system. In suspicion of urothelial malignancy of pelvis, she underwent a ureteroscopic biopsy which revealed malignancy of urothelial origin. The patient later underwent definitive surgery with radical nephroureterectomy, and immunohistochemistry of her nephrectomy specimen revealed poorly differentiated NET, which was positive for chromogranin A, synaptophysin and CD56 with a KI index of 25%. This case report highlights the importance of the indolent nature of disease along with diagnostic challenges requiring meticulous pathological examination and immunohistochemistry studies.
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