Renal lymphangiomatosis is a rare benign disorder caused due to lymphatic obstruction. This benign mesenchymal neoplasm is characterized by numerous intercommunicating cystic spaces containing lymphatic fluid. It can be focal/unilateral or diffuse /bilateral. Patient may be asymptomatic or may present with symptoms such as flank pain or may have renin dependent hypertension. Here we report a case of 32– year old female with focal renal lymphangiomatosis. The purposes of this study is to enumerate the radiological appearance of rare case of focal unilateral renal lymphangiomatosis.