Dedicator of Cytokinesis 8 (DOCK8) deficiency is a severe combined immunodeficiency that predisposes patients to recurrent and atypical infections. We report the case of an 8-year-old boy who presented with a rapidly progressive, fungating facial mass and keratomycosis, initially mimicking a neoplastic or mycobacterial process. An extensive diagnostic workup, including multiple biopsies, was necessary to identify Candida albicans as the causative pathogen. The severity and atypical nature of the infection prompted a comprehensive immunological evaluation, which revealed a biallelic pathogenic mutation in the DOCK8 gene. This case highlights the importance of meticulous microbiological and histopathological work-up and appropriate treatment of fungal infections with unusual clinical features

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