We describe an 11-year-old girl with severe renin-dependent hypertensi
on. She had characteristic cutaneous, ocular, and vascular signs of ps
eudoxanthoma elasticum (PXE), and the diagnosis was confirmed histolog
ically. Because PXE is a heterogeneous heritable disorder, the family
was evaluated for this condition. Since the patient's mother and broth
er showed some manifestations of PXE, autosomal dominant inheritance i
s the most likely inheritance pattern in this family.