OBJECTIVE: To report a successful pregnancy in a patient with pure 46,XY gonadal dysgenesis. DESIGN: Case report. SETTING: Academic reproductive endocrinology and infertility unit. PATIENT(S): A patient with pure 46,XY gonadal dysgenesis and a desire to become pregnant. INTERVENTION(S): Laparoscopic gonadectomy, in vitro fertilization using donor oocytes, transfer of cryopreserved blastocysts, and cesarean delivery. MAIN OUTCOME MEASURE(S): Successful pregnancy and live birth. RESULT(S): Successful pregnancy and delivery of a healthy infant following in vitro fertilization using donor oocytes and embryo transfer. CONCLUSION(S): With the use of donor oocytes, patients with pure 46,XY gonadal dysgenesis can anticipate successful pregnancy.