Case report: This report describes clinical data of 6 Kuwaiti patients having HT1.The index case was the product of consanguineous marriage male presented at five months age with hepatic failure, died at age of 7 months. Based on this family history all following siblings were screened ,three children (two males and one female)were affected. The second index case (the cusion of the first index case) was a female died at age of 6 months with hepatic failure. Her sister was diagnosed on birth based on family history. Once diagnosis was confirmed, all patients were started on tyrosine free diet and NTBC 1 mg/kg/day twice daily. They were monitored regularly. Results: Patients are leading normal life, 2 males aged 15 and 9 and 2 females aged 13 and 8. Although they have poor dietary control, their tyrosine level ranged between 800-1000. None of the patients had any complication of the disease or the drug. Conclusion: Medical advances improved prognosis of patients with HTI. Screening of HTI is recommended in Kuwait. 3 1 National Children Hospital "OHMATDET", Kyiv, Ukraine 2 National Medical Academy of Postdiploma, Kyiv, Ukraine 3 Research centre for medical genetics, Moscow, Russian Federation