Cancer of the small bowel (SB) is rare. The most common SB malignancy is neuroendocrine, followed by adenocarcinoma. SB adenocarcinoma with overlapping neuroendocrine differentiation (NED) is very uncommon. The present case report discusses the case of a young African American female who presented with high-grade SB perforation and underwent urgent surgical repair. Histology of the tumor revealed jejunal adenocarcinoma with NED. To the best of our knowledge, this type of tumor has not yet been reported in the jejunum. Referring patients that present with insidious and nonspecific abdominal discomfort for imaging studies that are more sensitive to the SB could potentially identify tumors earlier, prevent the development of emergent gastrointestinal complications and offer a chance for cure. An overview of diagnostic tools for SB evaluation was provided.