Introduction: Lymphoedema results from impaired lymphatic transport leading to the pathologic accumulation of protein-rich lymphatic fluid in the interstitial space, most commonly in the extremities. Primary lymphoedema, a developmental abnormality of the lymphatic system, may become evident later in life when a triggering event exceeds the capacity of normal lymphatic flow. Clinical Picture: We present a 3-year-old nephrotic syndrome patient with an unusual localisation for primary lymphoedema. Treatment and Outcome: The patient was treated with conservative approach and she was cured. Conclusion: In this particular case, lymphoedema developed at an unusual localisation, which has not been recorded before.