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Until 1954, 513 cases of phenylketonuria were recorded in the literature.1 Since then many more cases have been reported, both from America and Europe. Interestingly, only one of the reported patients had Jewish ancestors;2 another Jewish patient is mentioned in a personal communication.3 Considering the rarity of this metabolic disorder in Jews, a preliminary report on several cases of phenylketonuria we have discovered in a family of Iraqui Jews seems justified. In June 1958, a 21/2-year-o1d boy with mental and motor retardation was referred to our clinic for evaluation.