Patient 13, a 37-year-old woman, developed an abdominal muscle rupture and was found to have an aortic aneurysm (Figure 2b-III) during her high school days, which ruptured at 32 weeks of gestation of her first pregnancy (Table 3). She underwent an aortoplasty following an emergency cesarean section, which saved her life with no sequelae but resulted in severe hypoxic ischemic encephalopathy in her daughter. During her second pregnancy, she was suspected to have vEDS because of skin translucency (Figure 2b-IV) and easy bruising in addition to the previous events. Her cultured skin fibroblasts showed a decreased amount of type III collagen production (29.5% of normal, Figure 3a), whereas mRNA-based direct sequencing of COL3A1 showed no pathogenic variants. She underwent an elective cesarean section at 32 weeks of gestation with no consequences for her or her son. Obstetricians noticed an extreme fragility in her uterine wall during the cesarean. The current NGS-based investigation revealed a heterozygous nonsense variant in COL3A1 (c.848T>A:p.Leu283*), which was not registered in gnomAD or 8.3KJPN. She began celiprolol therapy. Her daughter was bedridden with mild skin translucency and easy bruising, and her son had moderate skin translucency, easy bruising, and several occurrences of muscle rupture while playing football. Both were found to have the variant and her son has begun celiprolol therapy.