Multicystic Dysplastic Kidney is a developmental disease that results in a lobulated kidney of noncommunicating cysts and abnormal parenchymal tissue. Dysplastic kidneys are usually benign and often involute over time with conservative management. The second most common cause of palpable abdominal mass in a neonate, Multicystic Dysplastic Kidney can cause respiratory distress secondary to extrinsic compression. However, such cases are sparse. Here we present the case of an otherwise healthy term newborn with an exceptionally large MCDK requiring CPAP support and intubation. His respiratory distress improved immediately after nephrectomy. Communication about cases like this will inform management of future comparable cases.