The objective of the present study is to present a case of a 48-year-old woman with leiomyomatosis and multiple pulmonary metastases. The classification, pathophysiology, clinical signs, treatment and prognosis of this rare case are discussed. Leiomyomatosis is potentially life-threatening while patients with pulmonary metastases are usually asymptomatic and the condition is incidentally discovered. The treatment of leiomyomatosis is not standardized and many possible variations are under investigation. Still the prognosis is usually excellent.