Pleuropulmonary blastoma (PPB) is a dysontogenetic neoplasm of childhood that involves lung and/or pleura. There is an increased incidence of neoplasias and dysplasias among young relatives of children with PPB. Pathophysiologically, PPB evolves from a cystic to solid state over time. It is subclassified as type I (purely cystic), type II (both cystic and solid elements), and type III (completely solid). Type II and type III may be associated with metastasis, with the brain being the most common metastatic site. The absence of epithelial malignancy in PPB is a feature that distinguishes it from the adult-type pulmonary blastoma. The clinical presentation includes signs and symptoms associated with various respiratory disorders. To make a definitive diagnosis of PPB, an examination of the cystic fluid or solid tumor is required. Treatment for PPB consists primarily of surgery and chemotherapy. Nursing care is directed toward maintaining normal respiratory and neurological function, maintaining normal fluid and electrolyte balance, minimizing side effects associated with treatment, and providing education for the family.