Background: Brain and central nervous system (CNS) cancers make up about 3% of all cancers around the world and are more common among men than women. Present study aimed to evaluate the occurrence trend of brain and CNS cancers in Iran from 1990 to 2017. Methods: This study using global burden of disease (GBD) 1990 to 2017 data demonstrated the trend of incidence and mortality of brain and nervous system cancer in Iran. All-age, sex-based, age-standardized, age-specific (from 1990 to 2017), and age distribution of incidence and mortality rates of brain and nervous system cancer were estimated in Iran during 2017. Results: Both incidence and mortality rates of brain and nervous system cancer increased from 1990 to 2005 in Iran, Eastern Mediterranean Region, and global level. During 1990, incidence and mortality rates of brain and nervous system cancer were higher in women than men, but gradually both rates increased in men and exceeded women in Iran. From 1990 to 2017, the value of age-adjusted rate was higher than all-age rate of brain and nervous system cancer. The most incidence and mortality of brain and nervous system cancer from 1990 to 2017 occurred in age group of > 70 and the least values were seen in 15-49-year-old group in Iran. Conclusion: The brain tumor occurrence and mortality in Iran was higher than other Eastern Mediterranean Region countries. Incidence and mortality of this cancer has an increasing trend in Iran and this trend somewhat was independent from population aging.