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Objective: To evaluate the clinical characteristics, biochemical parameters and the distribution of HLA‐DQ genotypes among adult patients with celiac disease (CD) in Northwest China. Methods: This cross‐sectional study retrospectively collected clinical, biochemical, and HLA‐DQ genotype of patients with CD from a tertiary hospital in Xinjiang Uygur Autonomous Region, China between March 2016 and December 2021. Small intestinal biopsy and serum‐specific antibodies were used to diagnose CD. Results: Of the 102 CD patients, 63.7% were women (female: male = 1.76:1), and the mean age was 47.3 ± 14.7 years at diagnosis. Common gastrointestinal symptoms included abdominal pain (50.0%), diarrhea (39.2%), and abdominal distension (24.5%). While common extraintestinal manifestations were anemia (48.0%), osteopenia or osteoporosis (36.3%), and fatigue (35.3%). Approximately 34.3% of patients with CD had comorbidities, with the most common being thyroid diseases (18.6%). Biochemical profiles showed lower hemoglobin, higher platelet count, and 25‐hydroxyvitamin D (25[OH]D) deficiency. HLA‐DQ2/DQ8 was detected among all 53 patients who underwent genotype testing; the frequency of the HLA‐DQ2.5, DQ2.2, and DQ8 haplotypes was 71.7%, 24.5%, and 3.8%, respectively. Conclusions: CD was more common among women. Clinical manifestations include primarily gastrointestinal symptoms, but extraintestinal manifestations were not uncommon. Lower hemoglobin level, higher platelet count, and 25[OH]D deficiency are the main biochemical manifestations. The HLA‐DQ2.5 and DQ2.2 haplotypes are the most common genotypes in CD. [ABSTRACT FROM AUTHOR]