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Aim: Smooth muscle tumors are divided into leiomyomas and leiomyosarcomas. The aim is to present a patient with a rare tumor called smooth muscle tumor of uncertain malignant potential. Case report: The reported patient was a 48-year-old male, subfebrile, with chest pain. Chest computed tomography showed a sharply limited cystic formation of the posterior mediastinum. Esophagogastroduodenoscopy revealed outside compression in the middle third of the esophagus, and X-ray of the esophageal passage showed that the esophagus was pressed from the outside to the right and ventral. Bronchoscopy determined that the membranous part of the left main bronchus was slightly extramurally compressed. A left thoracotomy and an extirpation of the mediastinal tumor were performed. In pathohistology area of necrosis and tumor tissue composed of spindle cells were identified, which showed mild polymorphia. Immunohistochemical findings included positive smooth muscle actin (SMA) and desmin markers, negative S100 and CD34 markers, Ki-67 was 5.9%, and p16 was positive in more than 50% of tumor cells. The patient was followed up for 48 months, with no signs of tumor recurrence. Conclusion: Pathohistological diagnosis can be difficult, particularly in cases of rare neoplasms. The reported case shows that sometimes it *Dopisni autor: Prim. dr. sc. Veljko Flego, dr. med. Specijalna bolnica za medicinsku rehabilitaciju Thalassotherapia Crikvenica Gajevo šetalište 21, 51 260 Crikvenica e-mail: veljko.flego2@ri.t-com.hr 1Specijalna bolnica za medicinsku rehabilitaciju Thalassotherapia Crikvenica, Crikvenica 2Katedra za internu medicinu, Medicinski fakultet Sveučilišta u Rijeci, Rijeka 3Zavod za patologiju, Klinički bolnički centar Rijeka, Rijeka 4Zavod za pulmologiju, Klinika za internu medicinu, Klinički bolnički centar Rijeka, Rijeka Medijastinalni glatkomišićni tumor nepoznatog malignog potencijala: prikaz slučaja i pregled literature Mediastinal smooth muscle tumor of uncertain malignant potential: case report and literature review Veljko Flego1*, Darian Volarić2, Koviljka Matušan Ilijaš3, Ljiljana Bulat-Kardum4 Prikaz slučaja/Case report