AbstractThis case report describes the presentation, diagnosis, and treatment of a 13‐year‐old boy with pulmonary cystic echinococcosis. The patient presented with low‐volume hemoptysis, and lung imaging revealed a large cystic mass, as well as smaller pseudo‐nodular lesions, suggesting a large intrathoracic hydatid cyst and ruptured cysts. The diagnosis was confirmed by a positive echinococcosis Western Blot assay, despite equivocal serology. The treatment consisted of surgical removal of the large cyst using thoracoscopy, along with a two‐week course of albendazole and praziquantel, followed by albendazole alone for two years. Analysis of the cyst membrane revealed an Echinococcus granulosus protoscolex. The patient had a successful recovery.