Background: Vascular Ehlers-Danlos Syndrome (vEDS) is a rare connective tissue disorder that results from a gene mutation which encodes type III collagen. Clinical findings include skin hyperextensibility, joint hypermobility, and tissue fragility. Presenting signs in vEDS patients include arterial rupture, uterine rupture, and sigmoid colon perforation. Case Presentation: A woman in her 70s presented with nausea, five days of abdominal pain, and three days of obstipation. She had a known history of vEDS. Blood analysis demonstrated a leucocytosis (19) and elevated C-reactive protein (346). A computed tomography scan (CT) demonstrated severe gastric distention, dilated small bowel loops with a site of perforation identified, and small volume pneumoperitoneum. Pre-operative insertion of a nasogastric tube (NGT) was unsuccessful despite multiple attempts by staff. An emergent laparotomy revealed four quadrant enteric contamination with a perforation identified in the mid-small bowel. There was no evidence of ischemia. No clear cause for a perforation was identified, but overall tissue quality was poor. A NGT was inserted under direct laryngoscopy with Magill forceps, however an inadvertent laceration to the pharynx was sustained. Video fluoroscopic swallowing study (VFSS) and CT confirmed the pooling of contrast in the left parapharyngeal space which likely represented a traumatic false passage. The patient subsequently made a good recovery with resolution of the false passage on interval VFSS. Conclusion: Spontaneous small bowel perforation is an uncommon presentation in patients with vEDS. Invasive procedures in this cohort should prompt clinicians to consider NGT insertion under direct vision early should difficulty be encountered.