Only in the recent past has Prader-Willi syndrome (PWS) also been diagnosed in newborns and infants, and the first descriptions of respiratory disturbances in young, not yet obese patients have been published only recently. We report an infant with PWS and respiratory problems, who suffered sudden death. To our knowledge, this is the first such case. In order to avoid fatalities, it seems mandatory to monitor respiratory function in infants with PWS and to suspect PWS in all hypotonic newborns to determine the frequency of the syndrome more accurately, and, more importantly, to diagnose PWS at an earlier stage in order to take appropriate measures.

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