Echinococcosis is a zoonotic disease that can cause various human diseases, among which pulmonary echinococcosis is relatively common. This paper reports one case of pulmonary echinococcosis in a patient with pleural effusion as the main manifestation. The patient was a 70-year-old female admitted to the hospital due to "right upper abdominal pain accompanied by chest tightness for 16 hours". Chest CT examination indicated right-sided hydropneumothorax, right lung atelectasis, and pulmonary exudative lesions. The pleural effusion was dark red and turbid, and the Rivalta's test was positive. Microscopic examination showed a large amount of fibrinous necrotic material and fungal-like spores. Pathological examination after thoracentesis suggested possible echinococcal infection. Considering the patient's history of hepatic echinococcosis and residence in a pastoral area, and combined with the diagnosis from an external hospital, the case was ultimately confirmed as pulmonary echinococcosis. This disease commonly occurs in children and young adults in pastoral areas, and patients have an epidemiological contact history such as contact with infected dogs. The infection is transmitted orally through ingestion of food or water contaminated with echinococcal eggs. Clinical features often include cough, chest pain, and hemoptysis. Cyst rupture into the pleural cavity can cause hydropneumothorax, pleural effusion, and allergic reactions. This reported case was an elderly patient with atypical clinical manifestations, characterized predominantly by pleural effusion. The patient eventually died due to the infection. This highlights that similar cases in echinococcosis-endemic areas should receive high clinical attention, especially among elderly patients.