Giant splenic lymphangiomatosis in adult: a diagnostic dilemma.

Cystic lymphangiomatosis of the spleen occurs secondary to developmental malformation of the lymphatic system. It is one of the rare entities generally seen in children. It is usually seen in children. We report a case of cystic lymphangiomatosis of the spleen occurring in an adult woman presenting with massive splenomegaly. Total splenectomy was considered to be the treatment of choice for making definitive diagnosis and to exclude the presence of malignancy or other causes of massive splenomegaly.