INTRODUCTION Renal cell carcinoma is a common adult malignancy that can present incidentally or with a multitude of clinical symptoms and signs. Metastatic spread is frequent, occurring via haematogenous and lymphatic routes, although it does not typically present with lymphangitis carcinomatosa. CASE PRESENTATION We describe a patient who presented with cough and increasing dyspnoea. Initial chest x-ray and computed tomography were consistent with lymphangitis carcinomatosa that proved secondary to underlying renal cell carcinoma. CONCLUSION Lymphangitis carcinomatosa occurs with many different primary tumours and can rarely be the presenting feature of renal cell carcinoma. Underlying renal cell carcinoma should be considered in the differential diagnosis of lymphangitis carcinomatosa and excluded with subsequent investigations.