BACKGROUND Lipoid proteinosis (LP) is a very rare genodermatosis. The literature on LP consists of case reports only. As we have observed 14 LP patients belonging to nine different families in the last 15 years in our practice, we decided to review all reported Turkish LP patients in this 15-year period, and noted 37 diagnosed cases. The reasons for this relatively large number of cases, the clinical features of the patients, and the associations of LP with other clinical conditions are described in this article. METHODS Fourteen LP patients followed in our university clinic in Istanbul were scrutinized with regard to their demographic and clinical features. Diagnoses were established using clinical features, with histopathologic confirmation in 13 cases. RESULTS All but one of the patients had a history of consanguinity, or at least a marriage of parents from the same village. Typical cutaneous signs of LP and hoarseness of the voice were observed in all patients. Two patients of the same pedigree had insulin-dependent diabetes mellitus (IDDM), two patients from two different pedigrees had short stature, one patient had multinodular toxic goiter, and one patient had celiac disease. CONCLUSION LP is not rare in Turkey as consanguineous marriage is still a social problem, especially in some rural areas. The disease is not limited to a particular geographic region in Turkey. Short stature was observed in two cases from two different families, an association not reported previously; the association of LP with IDDM in one pedigree was thought to be coincidental.