Atraumatic splenic rupture revealing littoral cell angioma

Littoral cell angioma is a rare primary vascular neoplasm of the spleen. Spontaneous atraumatic splenic rupture is an exceptionally uncommon presentation and may result in life-threatening hemorrhage. We report the case of a 54-year-old woman with a homozygous factor V Leiden mutation who presented with acute abdominal pain and progressive anemia in the absence of trauma. Contrast-enhanced computed tomography demonstrated massive hemoperitoneum and splenic rupture without evidence of a focal splenic lesion. Owing to hemodynamic deterioration, emergency splenectomy was performed. Histopathological and immunohistochemical examination confirmed the diagnosis of littoral cell angioma. Owing to the rarity of this condition and the nonspecific radiological findings, diagnosis is frequently established only after splenectomy. Prompt recognition and urgent surgical treatment remain essential to achieve favorable outcomes.