Gastrointestinal stromal tumors (GISTs) represent rare neoplasms of the gastrointestinal tract. Here we describe a case with GIST and thrombocytosis presenting as an acute abdomen. Our knowledge, the co-existence of GIST and thrombocytosis has not been reported so far. A 66-year old female was admitted to the emergency room with epigastric pain and vomiting over duration of 3 days. Physical examination showed abdominal distension, rebound tenderness, and a palpable RLQ mass. The abdominal exploration showed that a 6x6x6 cm mass was located on small intestine. The mass was completely resected and enteroenterostomy was performed. Specific immunophenotype studies results showed GIST. During the post operative follow up, platelets were above normal levels 400x109/l. Therefore, bone marrow biopsy was performed and hiperplasia in megakaryocytes were found. The current treatment for localized disease is surgical resection. Co-existence of thrombocytosis and GIST has never been reported.

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