Pathological rupture of the spleen (PRS) is a rare, but well known complication of some hematological malignancies. In a recent review of the literature, Giagounidis et al. Identified 136 cases of pathologic rupture of the spleen since 1861. This number gives an idea of how seldom it occurs. In this review, 34% of the cases had occurred in acute leukemias, and 13% in acute lymphoblastic leukemias. In most cases, PRS occurs on the course of the disease. PRS as initial manifestation of ALL is a very rare feature: only six cases have been reported in the literature. Before now, in our hospital, we had only seen one case of a patient suffering from chronic myelomonocytic leukemia who presented splenic rupture as the initial manifestation of this disease. We now describe the seventh case, to our knowledge, of acute lymphoblastic leukemia presenting as pathologic splenic rupture.