Coats disease itself is a rather rare condition, whereas Coats syndrome has been observed in about 1.2-4.9% of all retinitis pigmentosa (RP) patients. The spectrum of clinical presentations and our therapeutic experience are illustrated with reference to case reports of three patients with clinically and electrophysiologically verified RP and Coats syndrome. As in classic Coats disease, Coats-like changes with RP are defined by aneurysmatic and teleangiectatic vessels and intra- and subretinal lipid deposits leading to exudative retinal detachment. Both entities differ with regard to age of onset, gender, bilaterality and location of the retinal changes. The reports published up to now show that there is no consensus concerning the success of the different treatments of Coats syndrome associated with RP. Laser therapy, cryocoagulation, diathermy and buckling procedures have been suggested for treatment of this complex. As in the therapy of Coats disease, our results confirm that the outcome of laser therapy depends on early detection of Coats-type vascular abnormalities and early initiation of the treatment. Therefore, thorough fundus examination should be regularly performed in all RP patients.