A Spanish boy was born at 34 weeks of gestation after a pregnancy complicated by polyhydramnios. Apgar score was 7/8, birthweight was 2600 g (50th percentile), length 44 cm (50th percentile), and head circumference (OFC or occipital-frontal circumference) 31 cm (50th percentile). The mother, a healthy 31-year-old, had a previous uncomplicated pregnancy producing a boy. Family history was unremarkable and there was no consanguinity. Physical examinations show that the infant had the appearance of severe premature aging. He had pseudohydrocephalus, large fontanelles, widely open sutures, and prominent scalp veins. He had a triangular face, with an "aged" appearance, micrognathia, a small mouth and nose, prominent eyes, and low-set dysmorphic ears. There was generalized absence of palpable subcutaneous fat and wrinkled, thin skin, which was more evident over the folds. Large, pedunculated nipples and cryptorchidism were present. The nails were normal. Physical examination at 4 months showed severe growth retardation with weight and length at <3rd percentile. The posterior fontanelle was large and alopecia of the scalp, eyebrows, and eyelashes was marked. His extremities appeared thin, with prominent stiff large joints (Figs.