Acute appendicitis is the most common cause of emergency surgeries, all over the world. Helminths like bacteria and viruses may cause acute appendicitis. However, superinfection of appendix with Enterobius vermicularis and Taenia spp. is very rare. Seventeen-year-old female patient, with abdominal pain underwent appendectomy, with preoperative diagnosis of acute appendicitis. On macroscopic examination, appendectomy specimen was hyperemic and edematous with fecalithes in the dilated lumen. On microscopic examination; proglottides and eggs of Taenia spp. and mature form of Enterobius vermicularis were identified in the lumen, with associated acute appendicitis findings. Here we report a very rare case of concomitant infection of appendix with Taenia spp. and Enterobius vermicularis, which is also the second case in the English literature according to our knowledge.