Leiomyosarcoma of the diaphragm is an extremely rare disease. No more than 20 cases have been described so far. All patients needed reconstruction of the dome of the diaphragm, including with the use of implants. The article presents a clinical case of treatment of a 40-year-old woman with giant leiomyosarcoma of the left diaphragmic cupola.The extirpation of the tumor as a single block with the diaphragm and the pericardial wall was performed, followed by the restoration of the abdominal obstruction with nickel-titanium metal knitwear.