Background: Mucormycosis is an uncommon life-threatening fungal infection. The major risk factors of this infection include uncontrolled diabetes mellitus, prolonged steroid therapy, persistent neutropenia, hematological malignancies, autoimmune disorders, trauma, burns and surgical wounds. Objectives: The current study aimed to determine the epidemiology and treatment outcome of mucormycosis in Khuzestan province, southwest of Iran. Methods: This cross-sectional study was performed during a period of 10 years from April 2004 to March 2014 at Razi hospital in Ahvaz, southwest of Iran, during years 2004 to 2014. Demographic data, laboratory data, clinical features, antifungal treatment, the need for surgical debridement and the outcome were collected. Data were summarized using descriptive statistical methods and analyzed by SPSS version 15 software. Results: The study included 20 patients with a biopsy-proven diagnosis of mucormycosis. Regarding the findings, the mean age was 51.4 +/- 9.7 years. Eighty-five percent of patients had uncontrolled diabetes mellitus. Findings showed that all the cases received amphotericin B, but surgical debridement was performed on 10 patients (50%). Most prevalent season of mucormycosis was winter (40%). Conclusions: Prognosis of patients that underwent surgery and medical therapy was significantly better than medical therapy alone (90% vs. 50% patient's survival).