Renal lymphangiomatosis is a rare, benign malformation, characterized by developmental malformation of the perirenal, peripelvic, and intrarenal lymphatics. Radiologist knowledge of the unique radiological features of this entity helps patient's safety in terms of management. We study the case of a 27-month-old boy presented to the emergency department with upper respiratory tract infection. He had a high blood pressure and had been diagnosed earlier with autosomal recessive polycystic kidney disease based on renal ultrasound findings. Because the clinical presentation and laboratory work of the patient did not support the diagnosis of autosomal recessive polycystic kidney disease, further, extensive work-up was performed, which confirmed the diagnosis of renal lymphangiomatosis. This case report emphasizes the imaging features of this rare entity to promote early diagnosis and better patient care.