AbstractBannayan‐Zonana syndrome is an autosomal dominant condition that has not been weii described in the dermatoiogy literature. The typical case is characterized by macrocephaly, multipie angiomas, and muitiple encapsulated or infiltrating iipomas. As in other autosomai dominant hamartoneopiastic syndromes, the degree of expression within one family frequently varies wideiy. Our patient had macrocephaly and angiomas as well as iipomas with peculiar histoiogic features simiiar to lymphangiomyomas. Her father had a iarge nevus flameus on his leg, and Iipomas with normal histoiogic appearance. The paternai grandfather had multiple encapsulated Iipomas with normal histoiogic appearance. Neither father nor grandfather had macrocephal