Objective To explore the clinical characteristics as well as diagnosis and treatment scheme of an adult patient with mucormycosis caused by Cunninghamella bertholletiae (C. bertholletiae) infection. Methods Clinical diagnosis and treatment process of an adult patient who was admitted to the department of hematology of a hospital due to invasive mucormycosis caused by C. bertholletiae infection were summarized. Relevant literatures were retrieved from databases for reviewing. Results A 54-year-old male patient was admitted to hospital due to "repea-ted fatigue for more than one year aggravated, and fever for one week". Pathological examination of lung tissue showed broad, irregular and undivided hyphae, thus morphologically identified as Mucor. Metagenomic next-genera-tion sequencing (mNGS) of alveolar lavage fluid and peripheral blood detected C. bertholletiae, thus patient was diagnosed as invasive mucormycosis caused by C. bertholletiae infection. Infection was controlled after treatment with liposome amphotericin B combined with posaconazole and caspofungin. 37 literatures were retrieved and 44 patients were involved (including the case in this study). There were 26 males and 18 females, with a median age of 52.5 (18-79) years. Underlying diseases were mainly hematological diseases (65.9%, n=29), and 14 cases underwent hematopoietic stem cell or solid organ transplantation. The most common sites of invasion were lung, brain and skin, with 36, 9 and 9 cases respectively. Number of cases with positive results of histopathology, fungal culture, direct microscopy and molecular detection were 28, 37, 29 and 17, respectively. 41 patients received antifungal therapy, 8 of whom underwent surgery. 30 cases died, with a mortality of 68.2%. The survival rate of patients receiving antifungal therapy combined with surgery (62.5%, 5/8) was higher than those with antifungal therapy alone (24.2%, 7/33). Conclusion Adult mucormycosis caused by C. bertholletiae infection is most common in patients with hematological diseases. Hematopoietic stem cells or solid organ transplantation are high-risk factors. The common infection site is lung and can invade multiple organs of the body, with high mortality. Pathogenic diagnosis is very important. Antifungal combined surgery can improve the survival rate.