A case report: a renal squamous cell carcinoma presenting as a nephrocutaneous fistula

Background Primary renal squamous cell carcinoma is a scarce entity, and its presentation as a discharging sinus is still exceptionally rare. We hereby report a case of a renal cell carcinoma in a young gentleman. Case presentation We report a case of a 20-year-old gentleman who presented with clinical features suggestive of left side xanthogranulomatous pyelonephritis (XGPN). He had undergone multiple endourological procedures for left side renal calculi in the past. We present the clinical presentation, diagnostic dilemmas and treatment for this patient. We performed an open radical nephrectomy for this patient. Our case report is unique from the point of view of nature of presentation to the final histopathological outcome in terms of age group of the patient. Here we present the clinical management and dilemmas in the management of this unusual and rare case. Conclusions Our case report and presentation has taught us that we as clinicians must have a high index of suspicion for a renal malignancy when treating patients who present with pyonephrosis associated with long-standing calculi diseases, irrespective of the age group of the patient.