BACKGROUND Glomus tumors (GTs) are rare mesenchymal neoplastic lesions derived from cells of the glomus body. GTs rarely occurs in the visceral organs, where there may be few or no glomus bodies, and the majority of GTs are benign, rarely demonstrating aggressive or malignant behavior and histological features. CASE SUMMARY We report a patient with malignant GTs of the intestinal ileum with multiorgan metastases who was admitted due to moderate anemia. Capsule endoscopy revealed a bleeding mass in the intestinal ileum, and the patient underwent segmental ileal resection through laparoscopic surgery. The histopathological and immunohistochemical diagnoses were consistent with malignant GT. Long-term follow-up showed that the GT had metastasized to multiple organs such as the colon, brain, and possibly the lung. CONCLUSION This case was characterized by the highest degree of malignancy and by multiorgan metastases, and it was the first case of intestinal GT uncovered by capsule endoscopy.