Systemic cystic angiomatosis is the involvement of multiple organ systems with a congenital vascular malformation. A combination of vascular anomalies, namely lymphangioma and hemangioma, can coexist. The liver, spleen, kidney, and colon are the most commonly affected organs. The clinical presentation varies and generally reflects the involved organ system. A case of systemic cystic angiomatosis involving the spleen, liver, and kidney is presented. The diagnosis and management during pregnancy is discussed. © 1990, Mosby. All rights reserved.