Paracoccidioidomycosis, the most prevalent systemic mycosis in Latin America. In children the most common clinical presentation is the juvenile or acute/subacute form, which mainly involves the lymph nodes and in a lesser proportion the gastrointestinal organs. We present the case of a 10-year-old patient, without comorbidities, who presented acute right iliac fossa pain; in addition to a history of 1 month of evolution of diarrhea, fever, weight loss, and hypereosinophilia. Was diagnosed of acute appendicitis due to systemic paracoccidioidomycosis, through histopathology of the cecal appendix and mesenteric lymph nodes. Although systemic paracoccidioidomycosis with involvement of the cecal appendix is ​rare, it should be considered as part of the differential diagnoses in children and young adults with generalized lymphadenomegaly from endemic areas.