BACKGROUND: Association of scleroderma with hemiatrophy is rare. The case we describe is unusual because of the combination in the same patient of several sub-types of scleroderma with oesophageal involvement and facial and extra-facial hemiatrophy. CASE REPORT: A 38-year-old women suffering from plaque-type morphea presented oesophageal dysfunction during the course of her disease with positive anti-Scl70-antibodies and progressive right-sided hemiatrophy of the face, sternocleidomastoid, thumb and thenar eminence. Linear hyperpigmentation of the right arm and a "coup de sabre" appearance on the face were also noted. DISCUSSION: Facial and extra-facial hemiatrophies are usually associated with or originate secondary to linear scleroderma. Only two cases with systemic involvement have been reported but hemiatrophy was localised to the face. The present case is unusual because of its onset as morphea in plaque form, because of the oesophageal involvement and the additional association of morphea in a linear form and facial and extra facial hemiatrophy. The relationship between sclerodermic facial hemiatrophy and Romberg facial hemiatrophy is also discussed. CONCLUSION: The combination of several sub-types of scleroderma and facial and extra-facial hemiatrophy in the same patient may indicate that these entities actually represent different spectra of the same disease.