Wilms tumor (WT) occurring in adults is rare and even much more rarely found to coexist with pregnancy. Clinical outcome in adults is worse overall compared with pediatric patients with WT and is often misdiagnosed with no standardized protocols for care guided by high‐evidence clinical trials. We present a case of a 23‐year‐old woman diagnosed with WT who was found to be pregnant immediately following nephrectomy. Workup findings showed that she had disseminated disease but was successfully managed in a multidisciplinary team setting with modified intrapartum chemotherapy followed by postpartum chemotherapy. In low‐resource settings, management protocols for adult patients with WT can be individualized by multidisciplinary teams to leverage available resources for best outcomes.