Aim: Mastocytosis is a rare disorder characterized by pathologic mast cell degranulation. This paper aims to present the dental management of a seven-year-old girl diagnosed with mastocytosis, in a hospital setting, yet without general anesthesia. Case Report: A seven-year-old girl, diagnosed with urticaria pigmentosa, was referred to a dental practice. Her parents’ main concern was a possible allergic reaction to local anesthetics and antibiotics. It was decided to perform treatment in the operating theatre of a clinic without general anesthesia. Eight primary teeth were restored. The patient was monitored, and no complications occurred throughout the procedure. Follow-ups performed every three months for two years after treatment. Patient’s oral hygiene was improved significantly. Conclusion: Collaboration between medical experts, patient’s family and the pediatric dentist was crucial. The dentist must be aware of the medical history and potential triggering factors. Due to high probability for life-threatening emergencies, dental treatment in a hospital setting is recommended.

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