Glomus tumours are uncommon neoplasms usually arising in the dermis and subcutaneous tissues where glomus bodies are generally found. Occasionally glomus tumours can occur in extracutaneous sites such as the gastrointestinal tract, bone, genitourinary system and respiratory tract. Primary pulmonary glomus tumours are very rare (only 17 cases reported in the literature), and are often confused with other solid neoplasms such as carcinoids, hemangiopericytomas and tumours belonging to the family of Ewing's sarcoma/primitive neuroectodermal tumours. We present a case of a primary pulmonary glomus tumour originating in the right main bronchus with focal invasion of the submucosa in a 69-year-old man. Histological and immunohistochemical features are reported. The current literature is briefly reviewed, with special attention to differential diagnosis and malignancy criteria.