A seven-year-old boy was admitted to our pediatric clinic with weakness, diffuse muscular pain, and nonfebrile convulsion. At physical examination, he had hypertension (160/100 mmHg) and tachycardia without fever (180/min). He later developed agitation and abdominal pain. Laboratory pathological findings were hyponatremia and low urine density. Urine porphobilinogen was found positive which was highly suggestive of acute intermittent porphyria. After making differential diagnosis, appropriate treatment was started. Acute intermittent porphyria is a rare disorder, but must be considered as a clinical entity in hypertension clinics. Anahtar Kelimeler : akut intermitan porfiri, hipertansiyon, porfobilinojen, acute intermittent porphyria, hypertension, porphobilinogen