<p>Pediatric gastrointestinal basidiobolomycosis (GIB) is a rare fungal infection caused usually caused by <i>Basidiobolus ranarum. It</i> primarily affects individuals in tropical and subtropical regions. This study aims to present clinical, diagnostic, and therapeutic insights from a comprehensive bicentric retrospective case series in Saudi Arabia. We retrospectively analyzed pediatric GIB cases from two tertiary hospitals in Jazan and Riyadh, Saudi Arabia. Data included demographics, clinical presentations, diagnostic approaches, treatment modalities, and outcomes. Diagnosis was based on clinical presentation, epidemiological context, and histopathological findings, with or without microbiological workup and fungal isolation. In this series, 42 cases were included with about 64% of cases being male and 76% aged six years or younger. Most cases were from Jazan Province, a southwestern region with tropical climatic features and extensive agricultural activities. The bowel was the most affected organ (90.47%), followed by the liver (29%). The diagnosis was initiated by clinical suspicion and relied predominantly on histopathological findings, as fungal culture was rarely done or yielded positive. About 83% of cases responded to voriconazole monotherapy, while 33% required surgical intervention. Relapse occurred in two patients, and one had persistent infection, all with hepatic involvement. Notably, no mortality was observed in this cohort. This study highlights the importance of early recognition and antifungal therapy in achieving favorable outcomes in pediatric GIB. Voriconazole monotherapy is highly effective, and cases can become complicated when the liver is involved. Further studies are required to streamline diagnostic workflows and optimize treatment protocols, including identifying factors that influence relapse.</p>