Introduction: Spontaneous nephrocutaneous fistula is a rare manifestation of renal disease that can occur due to various etiologies, such as renal calculus, chronic pyelonephritis, stricture of the ureteropelvic junction, and renal tuberculosis (TB). An autonephrectomy with a nephrocutaneous fistula due to renal tuberculosis can be diagnosed quite late if it is not suspected. Case Presentation: We report a case of a spontaneous nephrocutaneous fistula with tuberculous autonephrectomy. A 40-year-old white male with recurrent flank pain and intermittent purulent drainage from his right flank region for the previous 14 years was admitted to our outpatient clinic. Fistulography and computerized tomography demonstrated a 51×60mmarea with a soft-tissue appearance that implied autonephrectomy of the right kidney, and a fistula tract with a 9mmdiameter between the skin and the retroperitoneal space. The patient was successfully treated with nephroureterectomy and excision of the fistulous tract, followed by antituberculous treatment. The pathological examination of the surgical specimen revealed chronic atrophic pyelonephritis, calcifications, and necrotizing granulomatous inflammation suggestive of TB. Conclusions: Urogenital TB is difficult to diagnose due to the lack of specific symptoms and signs. In the case of a nonfunctioning kidney without an obvious cause and a chronic spontaneous nephrocutaneous fistula, the possibility of associated renal TB should be kept in mind, especially in immunocompromised patients or in places where TB is a common health problem.