Pseudoxanthoma elasticum is an inherited disorder of connective tissue that is associated with numerous systemic manifestations including various cardiovascular abnormalities. We present a case of a 35 years old lady who presented with abnormal wrinkling, pigmentation and laxity of skin. She also had hypertension, peripheral vascular disease, angioid streaks and mitral valve prolapse. She was thoroughly investigated and managed accordingly. She was also educated to minimize the potential risks involved and advised regular follow-up.