The current study documents an unusual case of iris metastasis as the first sign of small cell lung cancer (SCLC) and reviews the relevant literature to increase understanding and awareness of this rare condition. A 59-year-old male chronic smoker presented with a painful and red right eye without any disturbance of vision. The patient also complained of associated chronic cough and anorexia. Upon examination, an exfoliative, pinkish-white lesion of the right iris was observed. A systemic work-up was performed, which incidentally detected metastatic SCLC in the lower lobe of the right lung and was confirmed by histopathological examination. A thorough evaluation demonstrated no organ metastases, except in the iris. Following the failure of conservative approaches, the eye had to be enucleated. The clinical features, treatment and prognosis of this condition are briefly reviewed and the results of contrast-enhanced ultrasound (CEUS) examination observed in this case are presented. Despite their rarity, iris lesions should be considered as possible manifestations of underlying malignancies. Imaging techniques, particularly CEUS, may aid the detection, diagnosis and monitoring of such lesions.