Introduction. We present a patient with a delayed rupture of the spleen following a mild abdominal trauma. For years, the patient was treated for hereditary massive splenomegaly with thrombocytopenia, without established etiology. Case Report. The initial non-operative treatment lasted seven days, after which the patient was readmitted to the Emergency Department with signs of intra-abdominal hemorrhage and underwent emergency open splenectomy. Even after pathohistological examination, the etiology of massive splenomegaly remained unknown. Conclusion. Conservative treatment is not recommended in cases of massive splenomegaly; thorough surgical observation in a tertiary care hospital with interventional radiology and a good multidisciplinary team is necessary, while splenectomy is a surgery of choice.
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