Male patients have an increased risk of fractures and develop a number of endocrine/metabolic impairments known to adversely affect bone health. The aim of this study was primarily to determine the occurrence of fragility fractures and the bone mineralization status (lumbar spine, hip and total body by dual X-ray absorptiometry) in 36 male patients affected with type 1 myotonic dystrophy and 13 male patients affected with type 2 myotonic dystrophy. Fragility fractures occurred in 15 type 1 and 7 type 2 myotonic dystrophy in non-classical osteoporotic sites, such as metatarses. Hip osteopenia was the most frequent finding, particularly in type 2 (n = 6) than type 1 myotonic dystrophy patients (n = 1), while osteoporosis was rare. Patients with type 1 myotonic dystrophy presented higher total body bone mass density than patients with type 2 myotonic dystrophy and healthy controls and lumbar spine was associated positively with the severity of the disease. Gonadic failure, with low testosterone and reduced INSL3 levels, visceral adiposity and insulin resistance correlated with reduced body mass index in both type 1 and type 2 myotonic dystrophic patients. A 32-year-old male presented in a tertiary care hospital on 10th November, 2016 with complaints of difficulty in walking for four years. The attendant further tells us that he used to walk normally at first but then he felt like losing balance and fell forward. The episodes progressively increased in frequency and for the last year, patient has been almost bed bound. However, he still managed to get to the bathroom but with the support of two people. A week ago, he tried to walk independently but fell down hurting his knee. We were told that the patient had developed stiffness and some sort of deformity in his hands which he then couldn’t work with at all. He was fully aware of what was being said to him and knew what to reply but was unable to utter words thus his speech was affected as well. The condition was diagnosed by the doctors as ataxia. He had no history of alcohol intake or of any significant feud intake, no history of fever, diarrhea or weight loss.