Abstract Background Glomangiomyoma is a rare histological variant of glomus tumour. Clinically, it mimicks as a haemangioma and is challenging to diagnose. Its occurrence in the neck of a child has not been previously described. Case presentation A 3 year old girl presented with the complaints of painless progressive neck swelling in the right side for one and half year. Sonography, computed tomography (CT), magnetic resonance imaging (MRI), CT neck angiography and fine needle aspiration cytology (FNAC) were suggestive of vacular malformation i.e. giant haemangioma or arteriovenous malformation. The mass was removed in toto under general anaesthesia without postoperative complications. The histopathology confirmed it to be glomangiomyoma with haemangiopericytoma like features. Conclusion It’s an extremely rare variant of glomus tumour and may be the first report of a glomangiomyoma in the neck of a child. Despite a rare entity, it should be borne in mind during differential diagnosis.