Neonatal appendicitis (NA) is a very rare surgical condition. The aim of this study is to once again draw attention to this subject by collecting our cases with NA and cases of NA reported separately in English-language literature over the period from 1901 to 2000. We performed a retrospective chart review of patients admitted to our hospital, with the clinical diagnosis of NA from 1990 to 2000. A survey of the English-language literature together with our own 7 cases revealed a total of 141 cases of NA during the period of 1901-2000. 128 cases had sufficient information for analysis. The patients are grouped and discussed according to these 3 time- periods: 1901-1975, 1976-1984 and 1985-2000. The incidence, etiology, and presenting signs and symptoms of appendicitis in newborns are discussed. Despite the similar perforation rates in the 3 time- periods (73%, 70%, 82%), mortality rate in NA has decreased from 78% in the 1901-1975 period, to 33% in the 1976-1984 period, and to 28% in the 1985-2000 period. A newborn baby presenting with continuous vomiting, refusal to feed, and, showing signs of pain through irritability, restlessness, sleep disturbance, and a distended abdomen; one should strongly suspect an abdominal disorder, perhaps appendicitis.