One of the most common causes of acute pancreatitis in children are medications. These include L-asparaginase, glucocorticoids and 6-mercaptopurine, which are widely used in the therapy of acute lymphoblastic leukemia (ALL). When L-asparaginase and glucocorticoids are administered together, blood triglyceride levels increase, which consequently further enhances the risk of pancreatitis. Therefore, acute pancreatitis is a common adverse effect of ALL treatment, present in 2.3-11% of pediatric patients.The aim of this paper was to assess potential risk factors, treatment outcomes and recurrence of acute pancreatitis in children with ALL. Based on the studies conducted, we found potential risk factors, other than the drugs mentioned above, to be the patient's age at diagnosis, obesity, the type of L-asparaginase administered, and the cumulative or peak dose of L-asparaginase or other drug used. The course of pancreatitis is usually mild to moderate, and the treatment is mainly symptomatic. Moreover, a successful treatment option may be octreotide. As children who have received less than 25 weeks of L-asparaginase therapy have presented with inferior outcomes, it seems reasonable to reintroduce this drug into ALL treatment after an episode of pancreatitis. The incidence of recurrent pancreatitis after re-treatment with L-asparaginase varies depending on the study. The outcomes for children who develop acute pancreatitis during ALL treatment are usually worse compared to children without an acute pancreatitis history, but the results remain inconclusive. In conclusion, acute pancreatitis remains a serious adverse effect of ALL therapy in children, which may result in worsening patients' outcomes.