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To determine the prevalence of 6R-Tetrahydrobiopterin (BH4) responsive phenylketonuria (PKU) in 53 cases of patients with various classification of hyperphenylalaninemia and PKU Excluding the BH4 deficient type referring to children's medical center in Iran (phenylalanine 360–2420 μmol/L), the single dose of 20 mg/kg (Kuvan®) and duration of 24 h was used. Results: Among the 4 different categories of mild hyperphenylalaninemia requiring treatment, mild, moderate and classic PKU, the BH4 responders were 90%, 35.7%, 5.6% and 0% respectively after 24 h. Conclusion: BH4 responsiveness is more prevalent in mild hyperphenylalaninemia and mild PKU patients in Iran.