A small number of patients with angiosarcoma present each year, even fewer of whom have their primary origin site in the lungs. As such, specific treatments are not well defined for this tumor type. We report that the combination of gemcitabine and docetaxel may be an effective regimen for the treatment of angiosarcoma, as illustrated by the complete radiological response observed. In this case report, we review the clinical characteristics, prevalence and treatment options for angiosarcoma. In particular, we review the potential pitfalls and important attributes that should inform diagnosis.