Cornual pregnancy is a rare form of ectopic pregnancy. We describe a case of cornual pregnancy suspected by two-dimensional ultrasonography (2DUS) and confirmed by three-dimensional volume contrast imaging in the C-plane. Three-dimensional power Doppler showed a particularly rich blood supply and two-dimensional color Doppler mapping demonstrated arteriovenous malformation (AVM). The feeding vessel originating from the right uterine artery and AVM were demonstrated with B-flow spatio-temporal image correlation (STIC) modality. Conservative management was chosen to preserve the uterus. Angiography confirmed the diagnosis of AVM; embolization with polyvinyl alcohol particles and embolization coils was performed through the right uterine artery until occlusion of the AVM and feeding vessels had occurred. Postprocedure color Doppler mapping and B-flow STIC modalities demonstrated absence of flow in the AVM. Serum human chorionic gonadotropin (hCG) levels gradually fell to non-pregnant levels during the ensuing 5 weeks. To the best of our knowledge this is the first report of cornual pregnancy with AVM. We demonstrate here the value of new three-dimensional ultrasound modalities in the diagnosis of cornual pregnancy and the use of embolization as an effective therapeutic option when conservative treatment with uterine preservation is desired.