Background: Twin pregnancies consisting of a triploid fetus with molar change and a healthy coexisting fetus is an extremely rare phenomenon. Triploidy rarely advances to the second trimester, most resulting in a miscarriage, termination or stillbirth. Only five cases of a triploid fetus and a healthy co-twin have been reported in literature. We present the first documented case of a live delivery of both triploid fetus and coexisting viable twin. Case presentation: A 23-year old woman, gravida 2, para 1 was admitted at 26+4 weeks gestation with DCDA twins and a shortened cervix. Sonography revealed polyhydramnios, an abnormal cystic placenta, and small aortic valve for the presenting twin. Emergency caesarean section was performed at 28 weeks gestation for cord prolapse. Twin A was born a live infant with ambiguous genitalia, syndactyly, epicanthic folds and cerebral cysts. Twin B was born a healthy live male. Histopathology revealed features consistent with partial hydatidiform mole, and cytogenetics revealed a diandric triploidy in twin A. Twin A survived until day 8 days at which supportive care was withdrawn. Conclusion: Twin pregnancies with a triploid fetus and healthy co-twin pose a significant challenge for the clinician given the range of antenatal complications. Historically, the management approach has been towards selective termination of the triploid twin before 20 weeks gestation to increase survival and decrease the risk of preterm delivery for the healthy co-twin. Our case is rare where both babies were delivered alive with a coexisting triploidy.