We report a rare finding of the coexistence of splenic hemangioma and progressive vascular malformation of the left lower extremity in a child. The lesion on the left calf was described as a vascular malformation in computed tomography and magnetic resonance. At the age of one year, the abdominal Doppler ultrasound was normal. The examination was repeated at the age of six years due to recurrent pain in the left hypochondrium and revealed giant multiple splenic hemangiomas. The girl underwent splenectomy at the age of 14 years. Histological findings demonstrated multiple cavernous hemangiomas. We present our case report regarding the diagnosis of spleen hemangioma and indications for surgical management in children.