BACKGROUND Autoimmune hepatitis (AIH) is a chronic inflammatory disease of the liver. Data on the disease remain scarce in the Southeast Asia region. This study was undertaken to assess the profiles of AIH in Brunei Darussalam. METHODS Nineteen patients with AIH treated at the hepatology clinic, RIPAS Hospital (up until December 2008) were reviewed. Demographic, laboratory, histologic, clinical, and therapeutic data of the patients were collected. RESULTS The median age of the 19 patients at diagnosis was 52 years (range 33-70) with a male to female ratio of 1:3.75. All patients were diagnosed with type I AIH. The prevalence rate of the disease was 5.61/100 000 and was higher in the Chinese than in Malays and Indigenous people. Commonly seen presentations were abnormal liver function (52.6%), icteric hepatitis (36.8%), and decompensated liver disease (10.5%). Histologically advanced fibrosis was found in 47.4% and cirrhosis in 21.1% of the patients. Immune-mediated diseases were present in 36.8%. In a follow-up for 31 months (range 0.25-102), three patients died, 2 had progressive liver failure and 1 had lymphoma. Complete biochemical response was seen in 75% of the patients, partial response in 12.5%, and no response in 12.5%. HLA DRB1*03 (DR3) was detected in 18.2% of the patients and DRB1*04 (DR4) in 45.5%. There were significant associations between HLA Cw7 (P=0.038) and DQB1*04 (P=0.007). CONCLUSIONS The data of the 19 patients were comparable to those reported in the literature. Most of the patients were found to have abnormal biochemistry. There were significant associations between HLA Cw7 and DQB1*04, but not between DRB1*03 (DR3) and DRB1*04 (DR4).