We report the unusual case of a child referred for persistent pneumonia and possible pulmonary nodules who was found to have a cardiac fibroma and diagnosed with Gorlin syndrome. Pulmonologists should be aware of the potential for vascular anomalies associated with this syndrome as well as the risk for pulmonary metastases from primary skin tumors. Pediatr Pulmonol. 2014; 49:E7–E9. © 2013 Wiley Periodicals, Inc.