The working career of 138 adult patients with autosomal dominant facioscapulohumeral muscular dystrophy (FSHD) was studied using a mailing questionnaire. Of 126 patients with an occupational history, the average female and male working career was 15 to 30 years, respectively. Of 61 respondents actually holding a job, only nine had adjustments for disease-related handicaps. Reaching above and below shoulder level was required in 24 jobs, although shoulder weakness is a presenting symptom of FSHD. In spite of these physical problems, 52 patients (85%) labelled their job as satisfying. The duration of their working career and the satisfaction with their work despite frequent physical problems warrant a careful vocational guidance of FSHD patients in order to prevent or postpone unemployment. Similar working career studies in patients with other neuromuscular disorders are worthwhile.