Acute infectious necrotizing fasciitis with external genital tract lesions and perineum (Fournier gangrene) refers to rare diseases that develop mainly in elderly people with burdened comorbid background, and as a rule, has a rapid course. The prognosis for this disease depends directly on the term the qualified surgical care has been provided. Delayed medical measures, even within just a few hours, lead to an increase in mortality reaching 88% under this pathology. In children, cases of Fournier gangrene are extremely rare and are represented in the literature by single descriptions. The article presents a clinical observation of surgical treatment of a 1-year-old child with Fournier gangrene. The clinical case is described with the purpose of acquaintance with the pathology rarely observed in childhood that can be met by every urgent pediatric surgeon or pediatric urologist. Perhaps, our approach to its diagnosis and treatment can help determine the diagnosis timely and make the right decision about surgical tactics.