BACKGROUND Ectopic molar pregnancy is a rare phenomenon and has not been reported in the presence of an intrauterine device (IUD). Clinical diagnosis of molar pregnancy is challenging and requires careful follow-up. METHODS A 25-year-old woman (gravida 2, para 0) with a copper IUD in place presented with a positive pregnancy test. Diagnosis of the complete hydatidiform mole was pathologically confirmed after surgery following clinical and sonographic investigations that identified a left-sided ectopic pregnancy. CONCLUSIONS Gestational trophoblastic disease (GTD) presenting as an ectopic pregnancy is a very rare occurrence. This patient recovered without event through a combined management and follow-up for ectopic pregnancy and gestational trophoblastic disease. Appropriate identification and management of this clinical problem is essential in order to prevent initial complications as well as subsequent malignant sequelae.