This case report describes a male patient aged 44 with recurrent hypotensive shock and severe pulmonary obstruction. The diagnosis of systemic mastocytosis was suggested by the presence of small pigmented maculae (urticaria pigmentosa) and intolerance to NSAIDs. The diagnosis was confirmed by microscopic investigation of the pigmented maculae and bone. The patient was advised to avoid certain foods and drugs and has so far been free of symptoms. In patients with hypotensive shock and (or) severe pulmonary obstruction without a clear cause, systemic mastocytosis should be included in the differential diagnosis.
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