Abstract
Hyperimmunoglobulin E syndrome or Job syndrome is defined as an immune and connective tissue disorder that results in complications such as invasive infections. On the other hand, Fournier gangrene is a rapidly progressive necrotizing fasciitis that affects the perineal area, has a polymicrobial etiology, and its treatment consists of rapid and aggressive surgical debridement, antibiotic therapy, and hemodynamic support. We present the case of Fournier gangrene in an 11-year-old boy with a diagnosis of hyperimmunoglobulinemia E. who was admitted with scrotal necrosis with favorable evolution after initial medical management.