Cystic lymphangioma is uncommon angiomatous tumor that mainly occurs in the neck. Less than 1% of these tumors affect the mesentery, retroperitoneum and greater omentum. In particular, the cystic lymphangioma involving the mesentery and the retroperitoneum is a rare lesion. We report here on an uncommon case of cystic lymphangioma that presented as a multilocular mass involving the mesentery and the retroperitoneum, and we also present a brief review of the relevant literature.