AbstractSpontaneous dizygotic unilateral twin tubal pregnancy is an extremely rare occurrence with a high risk for pregnancy‐related mortality, and a diagnostic challenge for obstetricians. We present a case of a 27‐year‐old woman with spontaneous twin tubal pregnancy. Transvaginal color Doppler sonography revealed 2 separate gestational sacs within the right adnexa, each containing an embryo. Cytogenetic examination of the trophoblastic tissues confirmed the diagnosis of dizygotic twin ectopic pregnancy. Clinical signs and symptoms together with imaging studies help in the diagnosis of this rare variant of ectopic pregnancies. © 2008 Wiley Periodicals, Inc. J Clin Ultrasound, 2009