Neonatal appendicitis is a rare entity, with a mortality rate that has decreased in past decades but remained high. Of cases reported in the literature, more than 50% occur in preterm neonates, and none have been diagnosed preoperatively. Here, we report the case of a female infant of 27-4/7 weeks' gestational age, who presented with perforated appendicitis on day of life (DOL) 30. She was thought to have medically refractory necrotizing enterocolitis (NEC), but was found, instead, to have perforated appendicitis during an exploratory laparotomy. A thorough literature search indicates she is the second youngest neonate to survive perforated appendicitis to date.