Background <p>Anorexia nervosa (AN) is an eating disorder that mainly affects adolescents and young women. The disorder is characterised by dietary restriction or purges leading to malnutrition with subsequent metabolic anomalies, changes in immune function and increased mortality for the most severe forms of the disease. The changes in immune function may lead to increased susceptibility to bacterial infections or, more rarely, fungal infections, where only seven cases of chronic pulmonary aspergillosis (CPA) and one case of invasive pulmonary aspergillosis (IPA) have been described in the literature.</p> Case presentation <p>Two cases of CPA (one male and one female) occurring in the context of extreme AN according to the DSM5 classification are described. These two cases are compared to eight cases (all females) described in the literature. Clinical, biological and radiological data were collected. The majority of the patients (9/10) were female. AN was considered to be extreme in seven patients, severe in one patient and undetermined in 2 patients (BMI missing). Preexisting lesions were mentioned in four patients and in our two cases. Computed tomography scans demonstrated the presence of endocavitary material in two cases in the literature. The presence of anti-<i>Aspergillus</i> antibodies (IgG and IgE) was reported in 6/10 patients. Galactomannan was detected in serum and bronchoalveolar lavage fluid in respectively 1 and 3 patients. An <i>Aspergillus</i> spp. was recovered from fungal culture in seven cases including: <i>Aspergillus niger</i> (<i>n</i> = 1), <i>Aspergillus fumigatus</i> (<i>n</i> = 5). Four patients underwent surgical removal of the mass and two of these were also treated with voriconazole (VCZ). Four patients received antifungal treatment alone, including VCZ monotherapy (<i>n</i> = 1), or a combination of antibiotic and antifungal with either VCZ (<i>n</i> = 2) or amphotericin B (<i>n</i> = 1). One patient received liposomal amphotericin B then oral voriconazole and another one received voriconazole before micafungin due to panazole-resistant <i>Aspergillus</i> strain associated with treatment for nontuberculous mycobacteria. Regression of the lesions was confirmed radiologically in two patients. The evolution was favourable for 8/10 cases. One death occurred in the context of IPA and the second after the patient left hospital against medical advice.</p> Conclusions <p>These observations illustrate the possibility of CPA, or more rarely IPA, in patients with extreme AN. Evolution is usually favourable and rarely fatal. This complication should be considered in a patient with extreme AN in the presence of a preexisting cavity. Management should be multidisciplinary, involving personalised refeeding, medical treatment of aspergillosis associated with surgical treatment depending on the clinical context.</p>