Occasionally, pheochromocytoma may manifest as cardiomyopathy, acute myocardial infarction, cardiac failure, and cardiogenic shock. Here we describe a case of a 37-year-old female patient with a history of diagnosed neurofibromatosis type 1 and hypertension with hypertensive crises. She presented with acute respiratory failure and was hospitalised to the intensive care unit of Tartu University Hospital with suspected pneumonia. During the treatment an opinion arose that the leading syndrome was cardiac failure for an unknown reason and cardiogenic shock. After the resolution of this life-threatening condition, patient’s hypertensive crises recurred. Diagnostic workup continued in the endocrinology unit where, based on the results of computered tomography, iodine-123 metaiodobenzylguanidine scintigraphy, and elevated urine metanephrine and normetanephrine level, the patient was diagnosed with a 4.5 cm diameter pheochromocytoma of the right suprarenal gland. After appropriate preoperative α-adrenergic blocking with doxazosine, a right adrenalectomy with complete tumour removal was performed. No perioperative complications occurred and the patient has recovered both clinically and biochemically. It should be emphasised that in patients with cardiomyopathy for an unknown reason, cardiac failure, or signs of acute myocardial infarction, presence of pheochromocytoma should be considered for differential diagnosis.