Bilateral renal lymphoangiomatosis

INTRODUCTION: Renal lymphangiomatosis is a rare congenital benign disease of renal lymphatic system, here we are presenting a very rare form of disease which is bilateral form. PRESENTATION OF THE CASE: A young adult presented to our clinic after being referred from primary care clinic with intermittent bilateral flank pain and no other symptoms after extensive radiological investigations diagnosis has been made and confirmed by radiological finding of disease. Active treatment usually preserved for complex cases and for the complications of the disease but in our patient as needed analgesia worked well in controlling his intermittent pain and his wish not to pursue any intervention. The vague presentation with initial imaging rising suspicion of renal tumor or complex renal cyst might cause psychological street on the patient, which our patient had, but reassurance after extensive radiological work up relive that's stress. DISCUSSION: Although it is very rare disease to be bilateral but wide variety of other differential diagnoses make importance of disease recognition and accurate diagnosis is the key. CONCLUSION: Renal lymphangiomatosis is a rare benign disease of renal lymphatic, which usually affect one side, but bilateral form is very rare form, which may raise the suspicions of genetic form of renal malignancy. Accurate diagnosis requires work up to role out malignant and other renal tumor, which require active surgical management.