Frequency and prognostic importance of extrapulmonary manifestations have been examined in 254 patients with intrathoracic sarcoidosis. A follow-up was carried out after a median of 27 years from diagnosis. All patients could be traced. Extrapulmonary manifestations were found in 119 patients (47%), mostly occurring within the first years after diagnosis of intrathoracic sarcoidosis; skin manifestations were most frequently seen. ECG-abnormalities were found in 35 patients (14%); in 17 cases in the form of sinus tachycardia. No excess mortality was found (80 deaths observed versus 66 expected). The causes of death were related to sarcoidosis in 41%. Only tachycardia was associated with an unfavourable prognosis and this association vanished after adjusting for decreased lung function. None of the other extrapulmonary manifestations were associated with an unfavourable vital prognosis. Erythema nodosum was of no prognostic significance concerning survival. In conclusion, extrapulmonary manifestations are frequent in patients with intrathoracic sarcoidosis but do not influence vital prognosis.