The nevoid basal cell carcinoma syndrome

The nevoid basal cell carcinoma syndrome is a rare and complex disease with multiple manifestations. As an example, we present the case of an eight-year-old girl with this syndrome whose initial complaint was acute abdominal pain secondary to torsion of an ovarian fibroma. Because of its multisystem effects, pateints with this syndrome can present to any of a number of medical or surgical specialists. Close attention to the family and past medical history and physical examination will alert the clinician to its presence, allowing for appropriate genetic counseling and serial screening for the development of malignancies and other complications.