Pazopanib is a multi-targeted tyrosine kinase inhibitor, which is indicated for use in patients with advanced renal cell carcinoma or advanced soft-tissue sarcomas. Although rare, interstitial lung disease has been reported as among the adverse sequelae of pazopanib therapy. We report the case of a 75-year-old man who developed interstitial lung disease during treatment with pazopanib for renal cell carcinoma with multiple lung metastases. The patient presented with dry cough and new-onset fatigue 3 months after initiation of pazopanib. He had mild hypoxia with bilateral ground-glass opacities on chest CT. He was treated with antibiotics for presumptive pneumonia, but his respiratory status rapidly deteriorated, and he required non-invasive positive pressure ventilation. He recovered on discontinuation of pazopanib and systemic steroids. Clinicians should recognise that interstitial lung disease can occur in patients who are undergoing treatment with pazopanib.