Osteopoikilosis (Osteopathia condensans disseminata) is a rare and usually asymptomatic sclerosing bone dysplasia of unknown origin. Familial clustering suggests a dominant inheritance. The observation of a 47-year-old woman lead to differential diagnostic considerations in view of the literature on about 350 cases. For 2 years the patient has been complaining about pain and stiffness of both hands with swelling of the fingers. Additionally, she remarked about bilateral paresthesias corresponding to the sensory innervation of the median nerve. Clinical examination revealed a sensory carpal tunnel syndrome and mild synovitis of the proximal interphalangeal joints with skin induration and limited flexion of the fingers. In addition, some finger and toe nails showed pitting and most fingers had scar-like linear skin alterations. Radiologic findings showed symmetric, well-defined, homogeneous sclerosing areas in spongy bone. The combination of symmetrical sclerosing bone densities, hereditary character, and associated skin and joint manifestations suggests the existence of a general connective tissue disease.