| Summary: | Background: A fistula between kidney and pleura is a rare entity, particularly in children. Fistulation is mostly iatrogenic, following percutaneous nephrolithotomy and has a reported incidence of <1%. In the absence of recent renal surgery the diagnosis may be hampered by a lack of suspicion. However, in Case of pyelonephritis with ipsilateral pulmonary symptoms fistulation between kidney and pleura should be considered.We present a pediatric case of a nephro-pleural fistula due to chronic pyelonephritis, as well as a review of the literature. We highlight that the etiology of the fistula may impact the effectivity of the treatment. Conclusions: Surgery-related fistulas mostly heal spontaneously with optimal drainage of urine combined with antibiotic treatment. In contrast, pyelonephritis-related fistulas (most common xanthogranulomatous pyelonephritis) frequently require additional debridement by (partial) nephrectomy.
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