A 17-year-old girl was admitted to our hospital with severe refractory hypertension evolving over approximately 4 years. Despite not having the resources to identify plasma-renin levels and using standard imaging techniques, a juxtaglomerular cell tumor was suspected and was histologically confirmed after surgical excision. This is a potentially lethal condition if left untreated and surgical excision is curative. The benign nature of the tumor is emphasized and its chemical, radiological and microscopic appearance discussed according to the literature. To the best of our knowledge, this is the first reported case of a patient surviving a cerebrovascular accident associated with a juxtaglomerular cell tumor.