Pneumorrhachis(PR), or epidural emphysema, denotes the presence of air in the spinal epidural space. It can be associated with a variety of etiologies, including trauma; recent iatrogenic manipulations during surgical, anesthesiological and diagnostic interventions;malignancy and its associated therapy. It usually represents an asymptomatic epiphenomenon but also can be symptomatic by itself, as well as by its underlying pathology, and rarely can be fatal. The pathogenesis and etiology of PR are varied and can sometimes be a diagnostic challenge. As such, there are no standard guidelines for the management of symptomatic PR and its treatment is often individualized. Here, we present a case of a 14-year-old boy treated for leukemia who developed this complication and whether chemotherapy related or not, it proved to be fatal for him. To our knowledge, this is the first case in the literature of this complication with acute lymphoblastic leukemia. Pneumorrhachis (PR), or epidural emphysema, indicates the presence of air in the spinal epidural space. It can be associated with a variety of etiologies, including trauma; recent iatrogenic manipulations during surgery, anesthesiological and diagnostic interventions; malignancy and its associated therapy. It usually represents an asymptomatic epiphenomenon but also can be symptomatic by itself, as well as by its underlying pathology, and rarely can be fatal. The pathogenesis and etiology of PR are varied and can sometimes be a diagnostic challenge. As such, there are no standard guidelines for the management of symptomatic PR and its treatment is often individualized. Here, we present a case of a 14-year-old boy treated for leukemia who developed this complication and whether chemotherapy related or not, it proved to be fatal for him To our knowledge, this is the first case in the literature of this complication with acute lymphoblastic leukemia.