Muscular dystrophy encompasses a group of disorders characterized by the progressive weakness of the skeletal muscles. These disorders are mostly inherited and have characteristic age and muscle group predilection. Lingual muscle involvement is an unusual feature in patients with the muscular dystrophy and helps in the differential diagnosis. We recently encountered a serving soldier presenting with complaints of bilateral ptosis and dysphagia of 5 years duration. Examination showed bilateral ptosis, percussion myotonia, generalized muscular atrophy including that of tongue muscles, and a characteristic hatchet facies. Investigations revealed elevated creatine kinase and myotonic discharges on electromyography leading to a diagnosis of myotonic dystrophy type 1. Muscular dystrophy has a varied presentation and can pose a diagnostic problem in clinical practice. We present the case to highlight the differential diagnosis of tongue atrophy in patients with muscular dystrophy.