An adequate use of coping strategies could help patients to deal with disease-related stress. The study aim was to explore coping behaviour in adult patients with severe haemophilia and its possible determinants. Coping was assessed through three basic dimensions (task-oriented, emotion-oriented and avoidance coping), using the short version of the Coping Inventory for Stressful Situations (CISS-21). Patients' scores were compared with Dutch working men (N = 374), according to three categories: low use (<P25 of normal), average use (P25–P75) and high use (>P75). Determinants were measured using questionnaires on activities (Haemophilia Activities List), participation (Impact on Participation and Autonomy Questionnaire), physical functioning [physical component of the Dutch Arthritis Impact Measurement Scales-2 (D-AIMS2)] and socio-psychological health (psychological component of the D-AIMS2). In total, 86 adults with severe haemophilia (FVIII/IX<1%) were included. The median age was 38 years (range: 18–68) with 85% affected with haemophilia A and 75% using prophylaxis. Patients with haemophilia used task-oriented coping as frequently as the control group (P = 0.13); but used significantly less emotion-oriented coping (57% vs. 25%, P < 0.05) and avoidance coping (P < 0.05). Emotion-oriented coping showed a strong correlation with socio-psychological health (r = 0.67) and weak correlations with participation (r = 0.32) and social interaction (r = 0.29). Other associations of coping strategies with patient characteristics of health status could not be demonstrated. Overall, patients predominantly used the task-oriented approach to deal with their disease; the use of this strategy was comparable to the control group. Having a poor psychological health, less social interaction and/or less participation in daily life was associated with an increased use of emotion-oriented coping.