A 25-year-old male student complained about episodic palpitations, dizziness, nausea and headache 5 years prior to presentation. No otorhinolaryngic, neurologic or gastrointestinal causes were identified. Several ECG recordings revealed sinus node dysfunction with intermittent sinus arrest and AV-nodal escape rhythm. The patient was given a permanent DDD-pacemaker. Six months later, the clinical symptoms were unchanged. During an attack, physical examination revealed paleness, diffuse sweating and an arterial blood pressure of 250/130 mmHg, which decreased to 120/80 mmHg within a few minutes. Abdominal ultrasound and abdominal computed tomographic scan demonstrated the presence of a large (6.4 x 5.5 cm) left-sided adrenal mass. Two 24-h-urinary collections demonstrated elevated noradrenaline (mean 315 micrograms/24 h, normal < 80 micrograms/24 h) and adrenaline (mean 268 micrograms/24 h, normal < 20 mg/24 h) levels. Blood samples, which were drawn during excessive blood pressure rise, revealed elevation of plasma catecholamines (6.793 pg/ml for adrenaline (normal 50-150 pg/ml) and 10.424 pg/ml for noradrenaline (normal 200-500 pg/ml), so that the diagnosis of pheochromocytoma was considered established. The tumor was successfully removed during laparascopic surgery. After surgery, the patient remained well and normotensive. Three months later, several long-term ECG recordings showed sinus arrhythmia with no evidence of sinus arrest or AV-nodal escape rhythm, so that the DDD pacemaker was turned off. This case underlines that sinus node dysfunction with intermittent sinus arrest and AV-nodal escape rhythm is a potential early manifestation of a pheochromocytoma. These changes seem to disappear after successful removal of the tumor.