Alveolar rhabdomyosarcoma (ARMS) is more common in children and rare in adults. A small number of cases in the stomach of an adult have been documented. The present study describes a case of primary gastric ARMS in a 20-year-old healthy female. The patient was admitted to Handan Central Hospital (Handan, China) with intermittent abdominal pain for >1 month, which was noticeable and progressively worsened after meals, with black stools. Gastroscopy revealed a 2.5-cm ulcer on the side of the greater curvature of the gastric body, and a pathological biopsy revealed a neuroendocrine neoplasm. An enhanced abdominal computed tomography (CT) scan indicated a thickening and an ulcerated mass measuring ~4.5x2.0 cm at the gastric body, with the tumor showing poor enhancement. Finally, a laparoscopic distal gastrectomy was performed. The postoperative pathology combined with immunohistochemical staining indicated that the patient had primary alveolar RMS of the stomach. The present case suggests that an early diagnosis of gastric adenoid RMS is extremely difficult. However, this disease must be investigated in any young person who presents with a gastric mass. The current study presents a rare case of primary ARMS occurring in the stomach; however, more research on this disease is necessary to improve the clinical diagnosis and treatment.