Abstract: Background: Rhabdomyosarcoma (RMS) is a malignant tumor of striated muscle of mesenchymal origin regarded as the most recurrent soft tissue sarcoma in children and adolescents. We describe in this paper an unusual case of primary alveolar rhabdomyosarcoma of the stomach and review the literature. Case presentation : A 15-year-old girl presented with chronic gastric pain and vomiting evolving for 3 months. Computerized abdominal tomography detected enlarged celiac and lumboaortic lymph nodes. Gastroscopy revealed a friable cardial mass. Biopsies obtained from both the gastric tumor and the abdominal lymph nodes showed clear histopathological features of an alveolar rhabdomyosarcoma. Conclusion : Primary alveolar rhabdomyosarcoma of the stomach is extremely rare. Better awareness of this entity is necessary for early diagnosis and treatment.