A 15-year-old girl was operated for gastric leiomyoblastoma (epithelioid leiomysocarcoma) with metastasis to a regional lymph node. She has subsequently been operated on twice: once for local recurrence and metastases 9 years after her initial operation and once for an intraabdominal lymph node metastasis. Now, 13 years after her first operation for gastric leiomyoblastoma she is free from symptoms and signs of disease. Preoperative angiography was helpful in diagnosing the recurrence and planning the second operation. Our observations speak in favor of surgical treatment of recurrent and metastasizing gastric leiomyoblastoma. This patient was also operated for multiple pulmonary chondro-hamartomas, primarily suspected to be metastases. Coincidence of gastric leiomyoblastoma and pulmonary chondro-hamartoma does not seem to be due to chance alone.