A 36-year-old female patient visited the Department of Oral Medicine with the history of premature loss of multiple permanent teeth and a progressive shrinking of fingers for the past one year. Her past medical and surgical histories were not contributory. Family history revealed that she was the only child born out of a non-consanguineous marriage. She was of normal intelligence. Physical examination revealed short stature, coarse hair, bushy eyebrows and prominent forehead, mid-facial flattening, slight antimongoloid slant and short neck with scoliosis. She also had prominent epicanthal and nasolabial folds with the prominent, broad nose [Table/Fig-1a&b]. She had short stubby fingers of both hands [Table/Fig-2]. Oral examination revealed multiple missing teeth with resorbed alveolar ridges of maxilla and mandible. [Table/Fig-1a & b]: /b> Physical examination. Coarse hair, bushy eyebrows, prominent forehead, mid facial flattening, slight antimongoloid slant with prominent epicanthal and nasolabial folds [Table/Fig-2]: /b> Short stubby fingers of both hand Laboratory investigations, which included liver function test, kidney function test, thyroid function test, parathormone levels and urinalysis, were negative. As the patient was an orphan and had no other maternal relatives, genetic analysis was not performed. Hand wrist radiograph showed acrosteolysis of distal phalanges and subluxation at the proximal metacarpophalangeal joint of the middle finger and little finger [Table/Fig-3]. [Table/Fig-3]: /b> Hand wrist radiograph showing acrosteolysis of distal phalanges and subluxation at the proximal metacarpophalangeal joint of middle finger and little finger Lateral cephalogram showed an elongated ‘J’ shaped sella, hypoplastic frontal sinus and osteoporosis of cervical vertebrae with biconcave cervical vertebrae [Table/Fig-4]. [Table/Fig-4]: Lateral cephalogram showing an elongated ‘J’ shaped sella, hypoplastic frontal sinus and osteoporosis of cervical vertebrae with biconcave cervical vertebrae. In this picture blue arrow indicates hypoplastic frontal sinus, black arrow ... From the clinical and radiological features patient was diagnosed with Hajdu-Cheney syndrome. In most cases of HCS, treatment is based on neurological symptoms, but in this case the prognosis was good. Oral prophylaxis was done and the patient was advised to maintain good oral hygiene. To improve oral function the patient was referred to the Department of Prosthodontics for prosthodontic rehabilitation. The patient was started with oral vitamin D replacement therapy (6000 IU/day) and oral ibandronate 150 mg/month and was advised to have regular recall visits, for every three months.