Spontaneous remission (SR) in acute lymphoblastic leukemia (ALL) is a rare phenomenon, but the disease course and its underlying processes are of basic and clinical interest. Herein is reported the case of a pregnant, 31-year-old patient who developed ALL, followed by septic shock and SR of ALL. Information is summarized from earlier case reports and incidences of SR in ALL, to identify common patterns. Furthermore, the phenomenon of SR is compared with another disease variant of ALL, termed prodromal or preceding-ALL (pre-ALL). SR and the aleukemic phase in pre-ALL are associated with fever and/or sepsis and have similar kinetics and epidemiology. Therefore, pre-ALL not only closely resembles SR in ALL, but both conditions may represent a single disease entity. Production of pro-inflammatory cytokines and immune cell effects may induce temporary remission of ALL and the suppression of hematopoiesis. In contrast to SR in other types of cancer, all documented cases of SR in ALL were only transient. However, the disease can still be effectively treated with standard ALL therapies following relapse.