The MURCS association is a rare, nonrandom association of müllerian duct aplasia, renal aplasia and cervicothoracic somite dysplasia. The etiology is unknown. Although it is usually a sporadic disorder, familial cases with uterovaginal anomalies have been reported. Occasionally, it may be accompanied by abnormalities involving various other organs or systems. Malformations related to the central nervous system are very rare and the presence of seizures has not been reported previously. We present a 26-year-old female with MURCS association who had late onset, drug resistant partial seizures presumably originating in the frontal lobe.