Polymyositis is a rare disease entity belonging to the class of enigmatic disorders known as the collagen vascular diseases. It is characterized by proximal muscle weakness. More than 50% of the patients with this disease have development of head and neck manifestations, most commonly a heliotrope rash or dysphagia. Weakness and atrophy of neck muscles, and lolling of the neck have also been described. We report a case of polymyositis presenting as a neck mass, a heretofore undescribed manifestation of this disease in the head and neck. A review of the literature and an interdisciplinary approach to the diagnosis and management of this unusual disease will be described.