A neonate with a perforated appendix vermiformis in our neonatal intensive care unit (NICU) (case 1) prompted us to review our cases of exploratory laparotomy due to spontaneous intestinal perforation (SIP), surgical necrotizing enterocolitis (NEC), and other neonatal abdominal emergencies to identify additional cases of pathological appendiceal involvement (appendicopathy).A retrospective chart review was done for all the cases of exploratory laparotomy in our 16-bed community level-3 NICU between June 2003 and May 2014.A total of 34 patients with exploratory laparotomies were identified. Among the 11 cases with histopathology of the appendix available, only 3 cases had appendicopathy. Of these, only one was conclusive regarding the etiology.A perforated and/or inflamed appendix does not equal a diagnosis of true appendicitis. The term appendicopathy is in our opinion, therefore preferable because it can be either primary or secondary to more common diagnoses, such as NEC and SIP. The possibility of perforation of the appendix must be considered in all cases of appendicopathy but this diagnosis is difficult to make. Once appendicopathy is found, attempts should be made to identify its etiology to optimize treatment and prognosis. We thus propose an etiologic classification of neonatal appendicopathy.