Acute lymphoblastic leukemia (ALL) is a common malignant hematologic disease that is characterized by large numbers of dedifferentiated lymphoid cells. Statistical data of ALL's incidence and mortality are fundamental for policymakers to allocate resources optimally. In this study, we reported the incidence, death, and disabilityadjusted life year (DALY) of ALL in the globe from 1990 to 2017. Our analysis showed that the incidence case of ALL increased by 30.81%, while the age-standardized incidence rate (ASIR) maintained stable. Subgroup analysis by social-demographic index (SDI) showed that ALL's ASIR was significantly decreased in high SDI countries, but were moderately increased in high-middle SDI countries. The change trends of age-standardized death rate and DALY rate were similar to ASIR trends. Subgroup analysis by age groups showed that children and the elderly were more likely to suffer ALL. Risk factor analysis demonstrated that smoking was the most significant contributor to ALL's death and DALY in the globe. Besides, the high body-mass index is playing an increasingly important role in ALL-caused mortality. Multiple methods to counteract potential risk factors should be adopted, such as controlling body-mass index in all regions and avoiding occupational exposure to carcinogens in low SDI countries.