Background <p>The renin–angiotensin–aldosterone system is essential for blood pressure regulation, and its absence in children with bilateral kidney loss predisposes them to hypotension unresponsive to standard therapies. However, the longer-term hemodynamic consequences and neurologic risks in this setting remain poorly understood.</p> Methods <p>We report three pediatric patients with bilateral congenital or acquired kidney absence that developed prolonged hypotension unresponsive to multiple antihypotensive therapy. A systematic literature review identified seven additional cases. The clinical features, interventions, and outcomes are descriptively summarized for these 10 patients.</p> Results <p>All three patients exhibited hypotension lasting 6–28&#xa0;days despite preserved cardiac function without hypovolemia. In the combined literature review of 10 patients, the median age at hypotension onset was 7&#xa0;months (range, 1–48&#xa0;months). The etiology of kidney loss was congenital in 2 patients and bilateral nephrectomy in 8 patients. The median interval between kidney loss and hypotension onset was 14&#xa0;days (range, 0&#xa0;days–3&#xa0;years). Among the 10 patients, blood pressure normalized in 8, of whom 4 survived without neurologic complications. Four patients developed hypoxic–ischemic encephalopathy. Five patients died, including three with neurologic complications. Management strategies included fluid overload, vasopressors, and midodrine. Angiotensin II was associated with favorable outcomes in two cases.</p> Conclusions <p>Children with bilateral kidney absence can recover from prolonged hypotension; however, this condition is associated with an increased risk of neurologic complications. Careful monitoring and supportive strategies, including approaches reported to be associated with recovery such as Angiotensin II or fluid overload, may help minimize risk.</p> Graphical abstract <p></p>