Introduction Factor XIII deficiency is an extremely rare occurrence & a patient presenting with spontaneous rupture of the spleen is challenging to diagnose and manage. Case Report We present here a case report of a patient diagnosed to have factor XIII deficiency since early childhood, presenting with acute onset abdominal pain with hemorrhagic shock at middle age. On evaluation, the Patient was found to have spontaneous splenic rupture which was managed by emergency splenectomy with adequate blood products transfusion. Conclusion Factor XIII plays a major role in the final step of the coagulation cascade in stabilizing fibrin clot & factor XIII deficiency is a very rare inherited or acquired disorder. Manifestations of factor XIII deficiency vary from being asymptomatic to life-threatening hemorrhagic conditions. Patients may have to be dependent on repeated specific blood product transfusions in order to avoid bleeding diathesis.