Cutis marmorata telangiectatica congenita is a relatively benign, rare cutaneous disorder with various manifestations. A seven-year-old girl, who presented with extremity hypoplasia, had the characteristic reticular, patchy, blue-pink cutaneous lesions, which became more prominent with exposure to cold temperatures. She had 4.8 cm shortening of her right lower extremity, which was also thinner than on the left side. The patient did not have skin atrophy, ulcers, glaucoma or macrocephaly. She is being followed for a future extremity lengthening procedure.