Osteopoikilosis is very rare autosomal dominant disorder of unknown etiology which is found incidentally on radiological examination. It is also known as Albers-Schonberg disease or osteopathia condensans disseminata, characterized by the presence of multiple and often symmetrical radio-dense lesion in osseous tissue. Here we report a case of osteopoikilosis in a 30 years old man presented with left hip joint pain and restricted movements. Radiological study showed typical features of osteopoikilosis. Necessary investigations were done to exclude osteoblastic metastasis, tuberous sclerosis and synovial chondromatosis. The patient was treated with pharmacological and non-pharmacological approach with significant improvement of joint pain and movements.