The aim is to review the current evidence for the management of acute invasive fungal rhinosinusitis (AIFR) in immunocompromised children.

A systematic search of PubMed, Embase, and Cochrane Library was performed. Data regarding the epidemiology, clinical presentation, diagnostic instruments, and treatments of pediatric AIFR were narratively summarized and critically analyzed.

The management of AIFR in immunocompromised children can be very challenging because of the paucity of symptoms and rapidly progressive disease. The following key concepts are crucial to optimize the management: 1) early diagnosis through clinical features (sinonasal, oral, ocular, neurological symptoms and fever) and instrumental exams (nasal endoscopy with biopsy, microbiological tests, CT and MRI); 2) vigorous medical antifungal therapy associated with surgical treatment (endoscopic/open); 3) treat the underlying immunosuppression by improving immunocompetence; 4) close follow-up and prompt intervention in case of recurrence.

AIFR in immunocompromised children is a rare but serious condition that requires the use of fast and precise methods that can support clinicians in its management. In this perspective, a multidisciplinary diagnostic and therapeutic approach is essential. However, mortality is still high and further research is needed to improve knowledge in this field.