Severe sustained hypertension occurs in only 0.1% of the paediatric population and only about 2% of these patients will have an underlying endocrine cause. Phaeochromocytoma as a catecholamine-secreting tumour causing severe hypertension is exceedingly rare in children. A high index of suspicion and an awareness of the clinical spectrum are therefore necessary to make the diagnosis. Phaeochromocytomas can have protean manifestations which may be mistaken for a variety of clinical conditions. We highlight the problems encountered in making the diagnosis in an 11-year-old Chinese girl who presented with sustained hypertension, heart failure and transient renal impairment with two normal 24-hour urinary vanillyl mandelic acid (VMA) results before a third produced the diagnosis. We emphasize that total reliance on a single biochemical urinary screening is not acceptable. The measurement of urinary catecholamines or their metabolites increases the sensitivity of diagnosis. We recommend that in situations where biochemical screening is doubtful, appropriate imaging should be undertaken to exclude the diagnosis.