Cases of mixed infections of Mucor and Aspergillus in the lungs of children have not been reported. Here, we successfully treated and reported the first case of pulmonary coinfection with invasive fungal species in a diabetic girl. The patient was initially treated with a voriconazole-based regimen, but the infection only worsened. Subsequently, bronchoalveolar lavage fluid culture and mNGS were performed, leading to the clear diagnosis of simultaneous pulmonary infection by Aspergillus fumigatus and Rhizopus microsporum. Susceptibility testing revealed the resistance of the fungal strains to voriconazole. Therefore, we employed a combination of AmB liposomes and isaconazole to eradicate fungal infection effectively. This case demonstrates the significance of early and definitive identification of fungal pathogens, determination of effective antifungal medications, and timely administration of properly planned therapeutic regimens. Additionally, we conducted a comprehensive review of 10 cases of pulmonary Mucor and Aspergillus mixed infection, summarizing their characteristics and identifying commonalities.