Mucormycosis represents the third most common invasive fungal infection in children, and recent studies have suggested a rising incidence. Its case fatality rate is high, especially for neonates. Clinical presentation is influenced by underlying risk factors; associations with immunosuppression, neutropenia, diabetes, and prematurity have been described. It has been implicated in several hospital outbreaks. Diagnosis requires a high index of suspicion and evaluation with histopathology, culture, and, increasingly, molecular identification. Surgical debridement and antifungal therapies are the cornerstone for combatting invasive mucormycosis. However, the severity and relative rarity of this disease make comparative clinical trials for evaluating antifungal therapies in children difficult to conduct. Hence, therapeutic decisions are derived mainly from retrospective case series, in vitro data, and animal models. In this review, we summarize the literature on the epidemiology and diagnosis of this invasive fungal infection and provide suggestions on the management of mucormycosis in children.