Urticaria pigmentosa is the most common form of mastocytosis, a disease in which abnormal proliferations of mast cells occur in various organs of the body. Multiple stimuli, including many drugs commonly used in anesthetic practice, can provoke mast cell degranulation and result in intraoperative hemodynamic instability. An anesthetic plan minimizing histamine release and utilizing vecuronium as the muscle relaxant is discussed, and the literature pertaining to urticaria pigmentosa is reviewed.

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