Bannayan-Zonana syndrome is an autosomal dominant condition that has not been well described in the dermatology literature. The typical case is characterized by macrocephaly, multiple angiomas, and multiple encapsulated or infiltrating lipomas. As in other autosomal dominant hamartoneoplastic syndromes, the degree of expression within one family frequently varies widely. Our patient had macrocephaly and angiomas, as well as lipomas with peculiar histologic features similar to lymphangiomyomas. Her father had a large nevus flameus on his leg, and lipomas with normal histologic appearance. The paternal grandfather had multiple encapsulated lipomas with normal histologic appearance. Neither father nor grandfather had macrocephaly.