We present a one-year-old girl with maple syrup urine disease with dermatitis secondary to the restriction of amino acids as part of the treatment. We present the clinical evolution and histopathological correlation.

A male infant of 2,900 g was born at term to a 19-year-old primigravida woman who had adequate prenatal care and no major complications detected during the pregnancy. The only reported medical event was an episode of urinary tract infection by E. coli one month before delivering, which resolved without complications using nitrofurantoin. There was no history of maternal herpes simplex infection and her serologic screening was negative for syphilis.