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Spontaneous colon perforation is a known complication of vascular Ehlers-Danlos syndrome (vEDS) in the pediatric age group. The diagnosis of vEDS is often missed until the child presents to the pediatric emergency room with a complication like spontaneous arterial or colon rupture. These patients require immediate imaging and surgical attention to decrease morbidity and mortality. We present the case of a 9-year-old boy with abdominal pain and retching. An abdominal computed tomography revealed free air under the diaphragm, and surgical exploration showed perforation of the sigmoid colon. Ultimately, he was diagnosed with vEDS. We review the literature and discuss the clinical presentation, diagnosis, and life-threatening crises associated with vEDS.