The normal umbilicus is a simple structure, but the intrauterine development of the umbilicus is highly complex. Neonatal umbilical mass anomalies usually represent failure of obliteration of the vitelline duct or the allantois which results in persistence of remnants, which can lead to a wide variety of disorders. In this paper, we present a case of an appendicoumbilical fistula in a neonate along with the differential diagnosis and management options. Embryologic explanation of the etiology was discussed with the possible association with different forms of malpositioning and rotation of the gut.