Introduction: Contact of Paracoccidioides sp. with the human organism presents in a variety of clinical forms of the disease. Intestinal clinical manifestation is found in 10 to 30% of cases, commonly in juvenile form. This case report seeks to describe a patient with juvenile paracoccidioidomycosis with atypical intestinal involvement. Case report: A 14 years old woman with an updated vaccination schedule and no reports of chronic diseases, allergies or continued use of medications, presented a diarrheal condition that was characterized by pasty stools, where episodes became gradually more intense, associated with episodes of hematochezia, retroauricular lymphadenopathy on the right, as well as shivering and fever. A colonoscopy revealed multiple polypoid formations, ulcerations and friability throughout the colon. After biopsy results, the diagnosis of Paracoccidioidomycosis with intestinal involvement was established. Due to the wasting syndrome, anemia and persistent diarrhea, the patient was hospitalized for blood transfusion and intravenous treatment with conventional Amphotericin B. Discussion: Unlike what was presented by the patient in this case, involvement of the gastrointestinal tract is uncommon in paracoccidioidomycosis. This disease presents a range of clinical manifestations, which makes diagnosis difficult. Since this type of infection is not always considered, it may result in underdiagnosed cases, especially in women and young adults. The prognosis depends on the initial severity of the disease, the presence of comorbidities and the treatment used. Conclusion: Especially for the intestinal form, earlier treatment will better the results. The disease has a good chance of effective treatment using antifungal therapy.