The radiologic features of intralobar pulmonary sequestration (ILPS) have been describe and include the identification of a feeding systemic artery with venous drainage through pulmonary veins. Primary sequestration associated with typical hamartoma signs is really rare and has been described only once. We describe a patient with ILPS whose radiographic findings were unusual for two reasons. First, computed tomography (CT) demonstrated a bulky mass in the pulmonary sequestration. The size of lesion and histopathology made it an unusual presentation. Final histology study demonstrated pulmonary hamartoma with predominantly adipose and cartilage differentiation, which is an unusual complication originated from ILPS. Another sign also explains the second unusual feature, intracranial cholesteatoma, occurring concurrently with ILPS.
Keywords: Hamartoma; Pulmonary Sequestration; Syndrome.