Herein we describe a rare case of acute appendicitis associated with localized aspergillosis in an 8-year-old boy with acute lymphoblastic leukemia. During chemotherapy, the patient complained of mild abdominal pain in the peri-umbilical area and displayed an increased C-reactive protein level. Abdominal ultrasonography disclosed appendicitis and consequently an appendectomy was done. Histologically, acute appendicitis and Aspergillus hyphae were identified in the lumen and necrotic mucosa. However, there was no evidence of systemic aspergillosis. While aspergillosis is a common fungal infection in immunocompromised patients treated with chemotherapy, acute appendicitis associated with localized aspergillosis without systemic infection is a very rare occurrence.