Objective To analyze the hospitalization cost of the patients with hepatolenticular degeneration in southwest of China. Methods The data of hospitalization cost of the patients with hepatolenticular degeneration were acquired from electronic medical record system in West China Fourth Hospital from 2005 to 2020. Multiple linear regression analysis was utilized for analyzing the factors of hospitalization cost retrospectively, and covariance analysis was used for controlling the confounding factors. SPSS 20.0 (IBM) was applied for data analysis. Results A total of 346 patients with hepatolenticular degeneration were enrolled in our analysis. The year of admission, age, number of comorbidities, days of hospitalization, and complication of decompensated liver cirrhosis were the factors to influence the cost of hospitalization. After the confounding factors were adjusted, the hospitalization cost grew following the increase of comorbidity number, and complication of decompensated liver cirrhosis also led to the increase of hospitalization cost. The hospitalization cost of 2005-2006 and 2007-2008 were lower than those in the rest of years. Conclusions Hospitalization cost of the patients with hepatolenticular degeneration in southwest of China were influenced by number of comorbidities, complication of decompensated liver cirrhosis, and year to admit. It was important to reduce the occurrence of complication and decompensated liver cirrhosis, as to reduce the cost of hospitalization.