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Abstract Background and Objectives Zygomycosis, a severe form of fungal infection, is classified into two categories: Mucorales and Entomophthorales. Within the Entomophthorales category, Basidiobolomycosis is a rarely recognized genus that can have significant health implications. Prompt diagnosis and appropriate treatment, which includes the use of antifungal medication and surgical procedures, are vital for enhancing the prognosis of patients. The objective of this study is to investigate the response to treatment in patients hospitalized due to basidiobolomycosis. Methods We carried out a retrospective study, in which we analyzed data from 49 patients who were diagnosed with Entomophthorale, Zygomycosis, and Basidiobolomycosis at Namazi Hospital, Shiraz, between the years 1997 and 2019. The data included parameters such as demographic information, clinical symptoms, imaging findings, treatment methods, and patient outcomes. Results Out of 49 patients, 24 children, predominantly male (83.3%), were definitively diagnosed with basidiobolomycosis. The ages of the patients ranged from 1 to 16 years, with an average of 5.75 years. The most frequently observed clinical manifestations included abdominal pain (70.8%), fever (54.2%), hematochezia (41.7%), vomiting (20.8%), and anorexia (16.7%). Half of the patients exhibited failure to thrive (FTT), while abdominal distension was present in 25% of the cases, and a palpable abdominal mass was found in 37% of the patients. The primary treatment strategy incorporated surgical interventions complemented by a comprehensive antifungal regimen. This regimen included medications such as amphotericin B, cotrimoxazole, itraconazole, potassium iodide, and voriconazole. These were mainly administered in a combination therapy pattern or as a monotherapy of amphotericin B. Twenty-two patients were discharged, while two patients died due to complications from the disease. Conclusion Our findings indicate that the prevailing treatment modalities generally involve surgical intervention supplemented by antifungal regimens, including Amphotericin B, Cotrimoxazole, Potassium Iodide, and Itraconazole.