The aim of this study was to assess the distribution of human leukocyte antigen (HLA) groups in Turkish children with celiac disease (CD) and to investigate the association of HLA types and clinical manifestations of CD. Seventy-five children with CD were evaluated in two groups: Group I consisted of 45 classical celiac patients (15 males, 6.7+/-3.8 years); Group II consisted of 30 atypical celiac patients (9 males, 9.3+/-4.3 years). The control group consisted of 100 healthy renal transplantation donors. HLA typing was made serologically using standard lymphocytotoxicity techniques. HLA A29, B51, CW5, DR14, DR16, and DQ1 were the most common antigens in the control group. Frequency of HLA B13, CW7, B8, DR7, DR17 and DQ2 was higher in CD patients than in the control group (p<0.005, <0.05, <0.001, <0.001 and <0.001, respectively). The relative risks for HLA DQ2, B8, DR17 and B13 were 14.9, 13.6, 7.1 and 3.6, respectively. Frequency of HLA B35, DR11 and DQ7 was higher in classical CD than atypical CD, while a positive association was found between HLA B8 and atypical CD. A positive association was found between HLA B13, CW7 and DR17 in Turkish celiac patients in addition to HLA B8, DR7 and DQ2. This study also suggested that a correlation may exist between genotype and clinical manifestations.