Renal lymphangiectasia is one of the rarest surgical complications in post-transplant kidney patients. A few patients may clinically complain of nonspecific symptoms, and the other few might be diagnosed incidentally. We report the case of a 32-year-old female patient with a known case of Joubert syndrome who presented with nonspecific clinical manifestations. The patient underwent ultrasound, magnetic resonance imaging (MRI), and nuclear medicine (NM) imaging to confirm the diagnosis, which showed radiologic features of renal lymphangiectasia. Conservative medical management was delivered to the patient.