Introduction: This case report describes the diagnosis of a glomus tumor in the second toe of a 38-year-old female, followed by surgical treatment utilizing a transungual approach to preserve the nail. This study highlights the diagnostic challenges and surgical strategies to treat such tumors while preserving nail integrity. Patient concerns: Pain occurred once a week, but over time, it increased, and just before seeking medical attention, she experienced pain more than 5 times a day. The pain worsened when cold water touched her toe. Diagnosis: We observed a slight hump indicating nail plate deformity, and the patient exhibited severe pinpoint tenderness (positive Love test) in the affected area. Color duplex ultrasound was performed for further investigation, revealing a hypervascular hypoechoic nodule measuring 0.5 cm in size at the nail bed of the right second toe. Intervention: The surgery was performed under digital nerve block anesthesia using a modified transungual nail-preserving approach for the excision of the glomus tumor. Outcomes: The pain that was reported prior to the surgery has improved postoperatively, and the recovery has been uneventful without any other complication. Conclusion: This paper provides a comprehensive examination of a rare glomus tumor in the second toe, elucidating both diagnostic intricacies and treatment modalities. It emphasizes the dual necessity of achieving total tumor excision while also considering aesthetic outcomes. The insights presented herein are intended to serve as valuable guidance for clinicians confronted with similar clinical scenarios, underlining the delicate interplay between effective tumor management and the preservation of cosmetic integrity.