Detecting the characteristic cutaneous features can lead to an early diagnosis of the superior vena cava syndrome. These skin changes usually represent indirect dermatologic signs of an underlying malignant neoplasm; for most patients, this syndrome is the initial manifestation of their cancer. The most common cause is a bronchogenic carcinoma, especially the small-cell variety, but others include lymphomas, primary mediastinal tumors, and metastases to the mediastinal lymph nodes from extrathoracic primary tumors, especially breast cancer. Treatment of the underlying malignant neoplasm and relief of the obstruction produce prompt improvement in the dermatologic findings.