2 respiratory distress syndrome, and anemia. No gender was assigned, and all staﬀ was instructed to refer to the infant as “baby” or “infant.” Labs drawn on day 1 of life were as follows: repeat kary- otype 46, XY, testosterone 239 ng/dL (normal 37–198 ng/dL, premature infant male; Kaleida Health Laboratory, Buﬀalo, NY) and 17-hydroxyprogesterone 122 ng/dL (normal 124– 841, premature infant 26–28 weeks, day 4; Esoterix, Raritan, NJ). Additional labs to aid in the establishment of the DSD were not obtained because of the infant’s low hematocrit and small blood volume. Anatomical studies including vesicoureterogram were deferred due to severe IUGR. Weekly testosterone levels were drawn to identify the expected postnatal rise in testicular testosterone at which time the plan was to obtain testosterone/dihydrotestosterone levels to address the diagnostic possibility of 5α-reductase deﬁciency. This strategy was devised by the team to maximize the likelihood of making a diagnosis at the time of testosterone surge while limiting blood draws. On day 38 of life, the infant developed presumed sepsis and expired. At the time of death, no gender had been assigned, and further testing to evaluate for 5α-reductase deﬁciency could not be performed as testosterone levels remained <20 ng/dL. 2.1. Clinical Case Management. On day one of life, the pedi- atric endocrinologist met with the parents and advised that gender assignment would be deferred while the diagnostic evaluation was pursued. The parents were informed that their child would be referred to as “baby” or “infant” until gender was assigned. In addition, they were told that a psychologist experienced in helping families and children born with these conditions would be contacted immediately and would meet with them. On day 2 of life, the psychologist met with the parents for the purpose of (1) reinforcing the recommendations of the medical team, (2) describing typical development of the sexual-reproductive system and how their baby was diﬀerent, (3) agreeing to serve as a liaison between the family and the medical team, and (4) assuring them that if they decided on a gender diﬀerent from the team’s recommendation that this would not compromise their child’s care. The parents were engaged as an integral part of the decision-making team. The parents requested that all information relevant to gender assignment be given to them in composite rather piecemeal and decided that the psychologist would be the team member to relay information to them. At session two on day 11 of life, the psychologist reviewed the information previously discussed and presented a detailed description of the DSD diagnoses under consid- eration. The parents were informed of the tests necessary to discriminate between diagnoses and the reasons that the team was unable to obtain all of the needed blood for testing. They were informed that a stepwise approach to diagnostic testing would be needed because of their child’s medical condition. The parents agreed to delay gender assignment, with the team keeping open the possibility of either a male or female gender assignment. Prior to the third session, the psychologist organized a meeting with the medical team to discuss the parents’ Case Reports in Medicine requests, review the general status of the infant, and discuss strategies to establish the DSD diagnosis. In addition to the psychologist, the team members at this meeting included the neonatologist, pediatric endocrinologist, and urologist. A systematic and stepwise approach towards diagnostic testing was developed taking into consideration that biochemical testing was limited by the infant’s size and hemodynamic status. Session three took place on day 17 of life and included the parents, psychologist, pediatric endocrinologist, and neonatologist. The family was briefed as to the medical status and concerns that the neonatologist had related to their infant, which included persistent anemia, hypotension, and respiratory distress. The parents were interested in clarifying the DSD diagnosis; understandably, at this stage in the infant’s admission, the parents were focused primarily on the medical condition of their child. The pediatric endocrinologist discussed the most likely DSD diagnoses (androgen insensitivity syndrome versus 5α-reductase deﬁ- ciency) based on the physical exam ﬁndings and biochemical studies to date. The endocrine testing necessary to aid in the DSD diagnosis was reviewed. The parents elected to pursue biochemical testing for 5α-reductase deﬁciency while delaying genetic testing for androgen insensitivity syndrome. The ﬁnal meeting occurred shortly after the infant’s death. The parents met with the psychologist, pediatric endocrinologist, and neonatologist. The purpose was to ﬁnalize the gender assignment by giving the baby a name and to review the preliminary autopsy report. Additional genetic testing was described that might clarify the DSD diagnosis and aid in describing future risk for the family. The family elected to assign a male gender to their child, indicating that this decision was based on the initial karyotype. They consented to further postmortem genetic testing to address the DSD. While no speciﬁc queries were made, it was the impression of the multidisciplinary team that the parents were satisﬁed with the team’s handling of this case. Mutational analysis performed to evaluate for androgen insensitivity syndrome demonstrated that there was no disease-associated mutation identiﬁed in exons 1–8 of the androgen receptor gene (GeneDx, Gaithersburg, MD). Interestingly, although the family consented to the testing, when they were contacted to review the results, they elected not to pursue further counseling. 3. Discussion Disorders of sex development (DSD) are a class of congenital conditions involving anomalies of the sex chromosomes, gonads, reproductive system, and genitalia. In certain DSD diagnoses, the external genitalia are phenotypically incon- gruous with the genetic sex. In such cases, gender assignment management is best handled by a team of experts [2]. The DSD presented here is consistent with an undervirilized 46, XY individual. The broad diﬀerential diagnosis in this cat- egory include rare causes of congenital adrenal hyperplasia (such as StAR mutations, 17-hydroxysteroid dehydrogenase mutations, 3β-hydroxysteroid dehydrogenase mutations),