During a post-mortem examination multiple pathological findings were discovered in an individual known to have suffered from Ehlers-Danlos Syndrome (EDS). The most prominent of these findings was severe curvature of the spine in both coronal and sagittal planes, suggestive of the rare Kyphoscoliotic Subtype of EDS (kEDS). Cardiac abnormalities included a rare quadricuspid pulmonary valve. Lung and bone irregularities, bilateral diaphragmatic hernias, and long segment bowel stricture were also identified. Immunohistochemical staining of skin, muscle, and lung tissue revealed atypical composition and the reduced presence of lysyl hydroxylase (PLOD1). Here we describe autopsy findings of a kEDS case and we discuss the implications of kyphoscoliosis on the heart and the lungs.