Recently, Aerococcus urinae, primarily recognized as a common pathogen in elderly women, has been reported to cause an extremely unpleasant odour of the urine in paediatric patients similar to trimethylaminuria (fish odour syndrome). Herein, we present a case of A. urinae urinary tract colonization in a 12-year-old otherwise healthy boy, who finally refused micturition outside from his home environment as a result of the unpleasant odour. Within the last year, three cases (including our own) of A. urinae colonization causing foul-smelling urine in healthy children have been published, suggesting that this condition might be as frequent as trimethylaminuria. In case of polymicrobial growth in a urine specimen, A. urinae as the leading pathogen will usually be missed by routine bacteriological investigation. Novel bacteriological techniques such as MALDI-TOF MS provide a rapid tool to recognize this pathogen in urine.
Conclusion: As treatment of A. urinae infection is simple, we recommend that in healthy children with malodorous urine, this pathogen is excluded before the initiation of costly metabolic investigations.