Case Reports in Medicine androgen insensitivity syndrome (complete or partial), 5α- reductase deﬁciency, ovotesticular DSD, gonadal dysgenesis, and single gene mutations related to testicular development (SRY, ST1, SF1) [2]. However, it is recognized that in as many as 50% cases of 46, XY DSD, no deﬁnitive diagnosis is made, with 30% of those having a codiagnosis of prematurity/IUGR [5]. Even amongst individuals with an established clinical diagnosis, the genetic and biochemical milieu is complex. In fact, in a study of individuals diagnosed clinically with either complete (CAIS) or partial androgen insensitivity (PAIS), androgen receptor (AR) binding was normal in 7% and 64% of CAIS and PAIS patients, respectively, and AR gene sequencing was normal in 17% (CAIS) and 77% (PAIS) patients for whom genetic mutational analysis was performed [6]. In addition, AR binding was normal in 3 of 69 individuals for whom exon screening of the AR gene revealed a mutation [6]. Based on the clinical presentation of our case, there was a high likelihood that a deﬁnitive diagnosis would not be established. However, in order to provide the family with the most complete information regarding long- term outcomes with respect to gender assignment, as well as risk to future children, the team pursued the biochemical and genetic testing necessary to establish a diagnosis for the family. In the presented case, the karyotype, elevated testos- terone level at birth, and presence of intra-abdominal testes are consistent with male sex; however, the limited virilization of the external genitalia virilization brings this assignment into question. In fact, if the prenatal karyotype had not been performed, this infant may not have been recognized as having ambiguous genitalia and may have been assigned female gender. Gender assignment is dependent on a number of factors including the diagnosis, the appearance of the genitalia, options for surgical intervention, potential for fertility, family outlook, as well as long-term treatment, and outcome-based evidence regarding satisfaction with gen- der assignment within diﬀerent DSD diagnoses [2]. It was reported previously that individuals diagnosed with 46, XY CAIS established an adult female gender identity [7]. How- ever, 3 recently reported cases of 46, XY CAIS individuals initially assigned as females self-changed gender to male [8–11]. These cases highlight the unanswered questions regarding the role of intrauterine brain androgenization and development of gender identity, particularly since eﬀects of prenatal and postnatal androgens are presumed to be absent in those with CAIS. Individuals with PAIS are dissatisﬁed with their gender assignment 25% of the time, regardless of the initial assignment either male or female [12]. A majority of individuals with 5α-reductase deﬁciency assigned female gender at birth transition to the male gender after puberty, and all those assigned male gender at birth live as males [13]. The outcome evidence related to satisfaction with gender assignment for other 46, XY DSDs is limited by small patient numbers and diﬃculties with long-term followup. In our case, diagnosis of DSD was signiﬁcantly hindered by the infant’s prematurity, extreme IUGR, and hemodynamic status. A stepwise diagnostic approach was taken to minimize the risk of compromising the infant’s clinical status, while, at 3 the same time, striving to arrive at a diagnosis that would aid the family in assigning gender [14]. 4. Conclusion We present a case in which management was carried out by a multidisciplinary team consisting of pediatric endocrinolo- gists, neonatologists, a pediatric urologist, a geneticist, and a psychologist specializing in the management of DSD. The psychologist served as the team leader coordinating communication between the parents and the medical profes- sionals. The team provides the family with a comprehensive explanation of normal and abnormal sexual diﬀerentiation which becomes a template upon which they can understand the DSD diagnosis and arrive at a decision regarding gender assignment. In addition, when available, scientiﬁcally validated outcome data is shared with the family regarding speciﬁc diagnoses [2]. In most cases, an infant born with a DSD is considered a medical “emergency”; every attempt is made to arrive at a medical diagnosis in a timely fashion. A lack of diagnosis is perceived as anxiety provoking for families and caregivers when faced with an infant for whom gender assignment is unclear. Based on our experience, we would argue that when information related to patient care is relayed in a consistent fashion, this anxiety may be alleviated. In our case, the decision to assign gender was deferred for over 5 weeks, with the ﬁnal decision (male) only being made by the parents upon the infant’s untimely death. This case teaches us an important lesson about the man- agement of disorders of sex development by demonstrating that, with support and education, gender assignments can be delayed while diagnostic workup is being pursued. We believe that this team-based strategy can be employed in other complicated medical cases in which a diagnosis or treatment regimen must be deferred. Given that the most recent consensus statement encourages the team approach as the standard of care for management of disorders of sex development [2], randomized studies to address whether parental anxiety is minimized by this method are unlikely to be feasible. Validated questionnaires to address the concerns of families related to the process of education, counseling, diagnostic testing, and decision making will be an important component to test our success in this ﬁeld. Data gleaned from clinical trials assessing parental stress and developmental stage at which children are diagnosed with disorder of sex development may guide the development of such question- naires in the future [15]. Abbreviations DSD: Disorders of sex development IUGR: Intrauterine growth restriction. Acknowledgment The authors have no duality of interest to disclose. They thank Laura M. Andolina for her assistance in this case.