Boerhaave syndrome is a condition described as spontaneous rupture of the esophagus that often results in mediastinitis, sepsis, and death. The authors report a case of Boerhaave syndrome in a child with acute lymphoblastic leukemia. At the time of diagnosis (10 days after rupture), the patient had an absolute neutrophil count of 0 and was treated with T-tube drainage of the perforation. The T-tube was removed 2 months after the operation. The patient has required three endoscopic esophageal dilatations of a mild esophageal stricture. He remains in remission from his leukemia, has normal growth and development, and is on a regular diet.