Mastocytosis is a disease characterized by abnormal mast cell proliferation in various tissues, especially in skin. Urticaria pigmentosa is the most common form of childhood cutaneous mastocytosis. A six-month-old girl presented with focal afebrile convulsion following facial flushing. Her trunk was covered with red-brownish maculopapular lesions. The Darier's sign was positive, and a biopsy confirmed the clinical diagnosis of urticaria pigmentosa. On follow up, a facial flushing episode and generalized convulsion were also noted. Electroencephalography showed paroxysmal abnormalities. This case is reported because of the rare coexistence of urticaria pigmentosa and afebrile seizure.

Keywords: urticaria pigmentosa, seizure.