Ergotamine, an ergot alkaloid, used for the treatment and prevention of migraine attacks, is considered as a teratogenic drug and, therefore, should be avoided in pregnancy. Here, we report a newborn infant with unilateral renal agenesis, urethral atresia, and pulmonary hypoplasia associated with the use of ergotamine for the treatment of migraine attacks at early pregnancy. Genitourinary anomalies in association with ergotamine usage were rarely reported and this was the third case of renal agenesia in association with ergotamine usage in literature. We suggest that ergotamine teratogenicity may be dose dependent and should be avoided in pregnancy for the possibility of genitourinary anomalies.