A giant renal angiomyolipoma (AML) in a patient with septo-optic dysplasia (SOD).

Angiomyolipoma (AML) is a rare benign renal tumor occurring in about 0.3 to 3% of the general population. Most frequently it takes the form of small single tumors occurring sporadically or accompanying tuberous sclerosis (Bourneville-Pringle disease). In some cases the tumor may reach a very large size and be a cause of various serious complications. This case description concerns a 26-year-old female patient, suffering from hypopituitarism, hypothyroidism and binocular blindness during the course of septo-optic dysplasia, in whom a giant, left renal AML was diagnosed and treated surgically. According to the authors' knowledge this was the first reported case of a huge size AML in a patient with de Morsier syndrome.