The association of appendicitis with schistosomiasis in the appendix is extremely rare in France. We report herein a new case in which the diagnosis was made, in the presence of pseudo-tumoral appendicitis, by frozen section biopsies. The patient presented with a typical acute appendicitis, without urinary symptoms, and with granulocytosis (14000 white cells/mm3, without eosinophilia). At laparotomy, the appendix was voluminous, with necrotic abscess, and lymph node masses were noted on the greater omentum. Frozen section biopsies of an omental tumor showed schistosoma eggs, without malignant cells. Appendicectomy and partial omentectomy were performed. Postoperative course was uneventful. Pathologic examination of the resected specimen showed schistosoma eggs in all layers of the appendix, and in the omentum. Final diagnosis was established by positive serology and by findings Schistosoma haematobium eggs in the urine. The patient was treated postoperatively by praziquantel. In conclusion, in case of acute appendicitis, with pseudotumorous appendix and lymph node masses, even with poor epidemiological findings on the clinical history, frozen section biopsies can sometimes establish the diagnosis of schistosomiasis with appendicitis, and avoid unjustified bowel resection.