A 24-year-old West Indian woman with a four-year history of systemic lupus erythematosus presented with progressive dypsnoea due to primary pulmonary hypertension. Despite showing a response to vasodilators, these failed to control the condition. Her pulmonary hypertension increased in severity, eventually resulting in her death. We believe primary pulmonary hypertension to be an unusual complication of systemic lupus erythematosus. We suggest that this diagnosis should be considered in all patients with systemic lupus erythematosus and progressive dypsnoea, as optimum benefit can only be obtained by early institution of vasodilator therapy.