Background <p>Patients with hereditary angioedema (HAE) exhibit emotional distress and a deteriorated quality of life. Emotional distress is associated with impaired emotional regulation, including alexithymia, which is characterized by difficulties in recognizing and expressing emotions. Our study assessed the presence of alexithymia in patients with HAE to determine whether patients with this disease experience difficulties and specific learning in emotion regulation. Thirty-nine adult patients with HAE answered a self-report questionnaire measuring: 1) alexithymia (<i>Toronto Alexithymia Scale</i>-20), 2) anxiety and depression symptoms (<i>Hospital Anxiety and Depression Scale</i>), 3) quality of life (<i>Angioedema Quality of Life Questionnaire</i>), 4) emotion regulation skills (<i>Emotion Regulation Skills Questionnaire</i>), 5) difficulties in emotion regulation (<i>Difficulties in Emotion Regulation Scale</i>), and 6) perceived quality of parental care (<i>Parental Bonding Instrument</i>).</p> Results <p>No alexithymia was detected in 39.5% of patients, 36.8% of patients had definite alexithymia and 23.7% had possible alexithymia. Alexithymia was associated with fewer skills overall and more difficulties with emotion regulation, as well as with more depressive symptoms and lower quality of life in terms of fatigue/mood. Emotion regulation skills and difficulties also appeared to be associated with overprotection in terms of perceived paternal style.</p> Conclusions <p>This study shows a low prevalence of definite alexithymia in HAE patients and non-deficient emotion regulation. The association between alexithymia and depressive symptomatology suggests the potential role of interventions targeting emotion regulation features</p>