Osteopoikilosis is a rare condition showing characteristic sclerotic lesions on radiographic examination, which are diagnostic for the trait. We report four patients presenting with various complaints and 49 members of their families who later were found to have osteopoikilosis. The mean age of all 53 was 27.5 years and the male:female ratio was 33:20. Most had lesions in the small tubular bones. We studied the epidemiological, clinical and radiological features of these patients and from the pedigrees conclude that the disease is inherited as an autosomal dominant.