Calcifying fibrous pseudotumor (CFP) is one of the most unusual benign tumors of childhood and is located mostly in soft tissues, pleura, and peritoneum. The first case of pulmonary CFP in a 7-year-old boy is presented. The clinicopathologic features of this rare entity are discussed with special emphasis on histopathologic features and prognosis including a brief literature review. There are no presumptive clinical and laboratory findings, including tumor markers and imaging techniques, that distinguish CFP from other space-occupying lung lesions. The most important diagnostic aid is to bear this entity in mind when a child presents with an unexplained pulmonary mass. Total excision is mandatory for the appropriate diagnosis and treatment. (C) 2004 Elsevier Inc. All rights reserved.