Littoral cell tumors (LCT) are rare primary splenic neoplasms, unique for their morphologic and immunolabeling features resembling the endothelial littoral cells lining the sinusoids of the red pulp. They include the more common and typically benign littoral cell angioma, as well as the less common, potentially malignant, littoral cell hemangioendothelioma (LCHE) and the aggressive littoral cell angiosarcoma (LCAS). The most common presentation of these neoplasms is splenomegaly, and diagnosis is made histologically following biopsy or resection. To better understand these tumors, a comprehensive, international literature search was performed. Patient and tumor data, including presenting symptoms, comorbid cancers, immunosuppressive states, splenic mass and tumor size were analyzed. Massive splenomegaly (≥ 1500 g) following splenic resection, which correlates with a splenic length of 20 cm preoperatively, was found to be significantly associated with the presence of malignancy in the LCT (P<0.05).