Splenic angiosarcoma is a rare and aggressive malignancy with an incidence of less than one per million and a fatality rate over 90%. Early diagnosis is of great importance for optimal management. Here, we report the case of a patient with splenic angiosarcoma who presented with prominent schistocytes, echinocytes, thrombocytopenia, and iron deficiency anemia, which in combination with radiographic evidence of a splenic mass, raised the suspicion for angiosarcoma and resulted in a prompt surgical intervention with curative intent. Resolution of the hematologic findings following splenectomy suggests that patients with this malignancy should be monitored for recurrent hematologic abnormalities as they may herald recurrence of the disease. We present a literature review on the hematologic manifestations that is associated with this malignant disease.